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pmc-6513966-1 | Case 1. A 50-year-old woman noticed masses in her left axilla and left supraclavicular area. She underwent biopsies of supraclavicular masses in another hospital and was diagnosed with poorly differentiated carcinoma (PDC) that was Villin positive. She subsequently received six cycles of chemotherapy as digestive tract tumors and exhibited cancer progression. The gene expression profile indicated that the metastases were more likely to originate from breast carcinoma. Subsequently pathology consultation showed PDC (IHC stains: AE1/AE3+, ER–, PR–, HER-2 0, GCDFP-15–, TTF-1–, and PAX8–). Tumors were subsequently controlled after changing to a regimen specific for TNBC. During follow up 15 months later, space-occupying lesions in the left breast were found, and a core needle biopsy revealed invasive breast cancer.
Comment. The patient had breast cancer presenting 15 months after her presentation with metastatic carcinoma; therefore, the diagnosis can only depend upon IHC. As IHC stains revealed ER–, PR–, HER−2 0, it is difficult to identify triple-negative breast cancer in metastatic cancer without a prior history of breast cancer. The 90-gene expression profiling of her initial biopsy predicted breast carcinoma. |
pmc-6513966-2 | Case 2. A 47-year-old woman noticed masses in her left lower neck and supraclavicular area. Ultrasound of the breast showed adenosis. However, no other space occupying lesions were identified by PET-CT. Pathology revealed squamous cell carcinoma (IHC stains: ER–, PR–, HER−2 0). The gene expression profile indicated breast cancer. Ultrasounds of the thyroid, breast, collarbone, and neck and axillary lymph nodes were added and showed a 22 mm *10.5 mm mixed echo focus in the left breast (BI-RADS 4C).
Comment. This patient has triple negative breast cancer that was confirmed by imaging examination of the breast. The imaging test and IHC stains were primarily non-diagnostic, and the 90-gene expression profiling of her initial biopsy predicted breast carcinoma, highlighting the organ that needed to be inspected. |
pmc-6514724-1 | A 48-year-old male presented with gross hematuria. Physical examination was unremarkable. Microscopic examination of the urine sediment revealed abundance of red blood cells. Serum analyses and ultrasonographic evaluation of the urinary tract were unremarkable. Contrast-enhanced abdominal computerized tomography (CT) scan demonstrated an 8-mm right distal ureteral stone, without ipsilateral hydronephrosis (). An initial trial of medical expulsive therapy was declared to have failed after 6 weeks. Based on CT attenuation of the calculus (1609 Hounsfield Units - HU), ureteroscopic surgery was favored over shock-wave lithotripsy (SWL).
Prior medical and surgical history including urinary system stone disease, were unremarkable other than a robot-assisted PN that performed two years ago for an incidentally detected renal mass. When the operative report and visual recordings were reviewed it was understood that after removing the renal mass, renorrhaphy had been performed in a single-layered fashion using a running suture. Renal parenchyma was compressed using Hem-o-lok clips at the renal capsule []. He had received blood transfusions in the early postoperative period due to clinically significant hemoglobin decline. Pathological examination revealed pT1a, Fuhrman grade 2, clear cell RCC with negative surgical margins.
The patient was placed in modified lithotomy position and an 8 F semi-rigid ureteroscope was introduced through the external urethral meatus. The right ureteral orifice was identified and a 0.035 inch/145 cm safety guidewire was introduced gently into the ureter up to the kidney. A second guide wire was inserted and the railroad technique was used to facilitate the passage of the endoscope along the ureteral lumen. The stone was visualized in the distal ureter () and fragmentation was initiated using Holmium laser. After disintegrating the cortical rim of the stone, Hem-o-lok clip became visible. Attempts to fragment the clip with the laser failed () and it was extracted en-bloc using the pincer forceps (). The ureter was inspected fluoroscopically and endoscopically, demonstrating no residual calculus or foreign body. After placing indwelling ureteral and Foley catheters, the operation was completed.
The duration of the procedure was 35 min. The postoperative course was uneventful and the patient was discharged home within 24 h after removing the Foley and indwelling ureteral catheters. At 3-month follow-up, urine and blood work-up were unremarkable and CT scan showed no residual calculus, collecting system dilation, renal mass or metastasis. |
pmc-6515007-1 | A 43-year-old male reported to endocrine clinic for evaluation of pancreatic lesions. His past history was relevant for total parathyroidectomy for parathyroid hyperplasia, with forearm autograph implantation at the age of 16 years. The patient has a strong family history of pancreatic and parathyroid disorders in his paternal grandfather, two paternal aunts, a paternal uncle, his father, and sister. Additionally, menin gene mutation was confirmed in his father and paternal uncle. The patient underwent a genetic testing at the age of 40 years and this confirmed menin gene mutation. Patient had no symptoms to suggest hypoglycemia, peptic ulcer disease, diarrhea, or other symptoms of endocrine disorders. Review of systems was unremarkable. On physical examination, the vital signs were normal and examination of the heart, lungs, and abdomen was also normal. He has no clinical features of hypogonadism or Cushing syndrome. The patient is married without children and does not smoke or drink alcohol. Additional blood test showed normal serum calcium, testosterone levels, FSH, LH, prolactin, and IGF-I. Additionally evaluation for Cushing syndrome was also negative. Serum gastrin, chromogranin A, vasoactive intestinal polypeptide levels, and 24-hour urine 5-HIAA levels were also normal, and a 48-hour fast did not confirm hypoglycemia. However, fasting serum pancreatic polypeptide level was elevated (520 pg/mL, reference 0-418). A CT scan of the abdomen showed multiple subcentimeter lesions. The patient refused any treatment initially; 11 months later the serum pancreatic polypeptide levels rose to 1198 pg/mL (range 912-1588), and 5 weeks later the value was 1215 pg/mL (). At this time a repeat CT scan showed 3 hyperenhancing lesions in the head and tail of the pancreas measuring 10, 9, and 4 mm (). These lesions correlated with 111–Indium Octreotide scan (). Since the patient refused any surgical treatment, he was offered octreotide treatment. Initially the patient was treated with the short-acting octreotide 100 μg 3 times daily, and this was later transitioned to a long-acting octreotide 20 mg monthly. Following the octreotide treatment, the serum pancreatic polypeptide levels normalized to 62 pg/mL in approximately 6 weeks (). A follow-up CT scan at 9 and 33 months later confirmed stability of the pancreatic lesions without evidence of metastasis. Additionally, the serum pancreatic polypeptide levels remained within normal limits (91.6 pg/mL) (). The patient continued to feel well and has tolerated octreotide without any adverse effects over the entire period of treatment. A most recent gallium-68 DOTATATE PET/CT scan () performed 3 years later, while the patient was on octreotide therapy, further confirmed the stability of the pancreatic lesions. Additionally, serum pancreatic polypeptide remained normal along with normal levels of somatostatin, glucagon, gastrin, vasoactive intestinal polypeptide, and chromogranin A during the entire course of octreotide treatment. |
pmc-6515025-1 | A 32-year-old multiparous woman was admitted at 37 2/7 weeks for induction of labor for abnormal antenatal testing. She had a history of a prior cesarean delivery for arrest of active labor at 7 cm and after extensive counseling chose to undergo a TOLAC. The initial cervical exam was 2 cm dilation and 50% effacement. Her labor was induced with oxytocin and amniotomy was performed at 5 cm dilation with clear fluid noted. She progressed from 2 cm to 5 cm over the course of 10 hours; interval time to progression to 6 cm was 3.5 hours. The patient then began to have a moderate amount of vaginal bleeding with associated minimal fetal heart rate variability and variable decelerations that did not improve with intrauterine resuscitation. She was taken to the operating room for repeat cesarean delivery due to concern for a uterine rupture and/or a placental abruption. Upon entry into the abdomen, there was no uterine rupture or dehiscence, nor was there evidence of a significant placental abruption. She delivered a male infant with a weight of 2659 g and Apgar scores of 9 and 9 at 1 and 5 minutes, respectively; arterial cord pH was 7.26. No extension of the hysterotomy was noted following delivery of the infant. Following repair of the hysterotomy, the bladder was noted to be distended and edematous, despite the presence of a patent Foley catheter. This finding prompted a vaginal exam to assist in evaluating the integrity of the lower uterine segment behind the bladder due to concern for an occult uterine rupture or dehiscence. The vaginal exam revealed a spontaneous detachment of the anterior rim of the cervix from approximately 9 to 3 o'clock. The avulsed portion of cervix appeared necrotic () and there was no bleeding noted at the site of cervical detachment. The cesarean delivery was completed, and an attempt was made to repair the site of the detachment vaginally, at which time the entirety of the cervix completely detached. No excessive bleeding was noted and placement of compression sutures at the site of detachment helped ensure hemostasis. Her recovery and postpartum course were otherwise unremarkable, and she was discharged on postoperative day #3. The patient was examined at a six-week postpartum visit. No cervix was seen on speculum exam and on digital exam the cervix was flush with the vaginal vault. The pathology report for the detached cervix showed diffuse hemorrhage and vascular congestion; there was no pathologic evidence of placental abruption. |
pmc-6515025-2 | A 22-year-old multiparous woman with a history of primary sclerosing cholangitis complicated by portal hypertension, esophageal varices, and thrombocytopenia was admitted at 29 0/7 weeks for evaluation and management of shortness of breath and lower extremity edema. Her obstetric history was significant for prior spontaneous preterm birth with placement of an ultrasound-indicated McDonald cerclage at 20 1/7 weeks of gestation in the index pregnancy. At the time of cerclage placement, the 5 mm Mersilene (polyester) tape was noted to be suboptimally placed along the posterior rim, distal to the internal os, so a second McDonald cerclage of 0-polyester suture was placed cephalad to the tape. She was admitted at 28 0/7 weeks for preterm labor and the cerclages were removed with no remarkable anatomic irregularities. She received antenatal corticosteroids and magnesium sulfate during her admission for preterm labor and was discharged on hospital day #3 with a cervical exam of 1cm dilation and 70% effacement. During her readmission at 29 0/7 weeks for shortness of breath, she began to have contractions and moderate vaginal bleeding on hospital day #3. Her cervical exam was 4 cm and 90% effaced. She was transferred to labor and delivery, where she continued to have a rapid cervical change to 6 cm. A brief fetal heart rate bradycardia occurred with spontaneous recovery to a normal baseline. At this time, findings were concerning for placental abruption or possible coagulopathy from decompensated liver failure with a total estimated blood loss of 500 mL. Amniotomy was performed to expedite vaginal delivery. However, cervical dilation did not continue and complete cessation of vaginal bleeding was noted. Careful visualization of the cervix revealed that the posterior rim of cervix was detached and traversing the presenting fetal head creating a tension band that prevented further dilation or descent. This band of cervical tissue was clamped and suture-ligated followed by rapid delivery. She delivered a male infant with a weight of 1325 g and Apgar scores of 5 and 7 at 1 and 5 minutes, respectively; arterial cord pH was 7.33. Following delivery of the placenta, vaginal exam revealed a partial posterior cervical detachment from 2 to 9 o'clock. The avulsed portion of the cervix did not appear necrotic and was bleeding briskly. Hemostasis was achieved with a single running locked suture. Her postpartum course was otherwise unremarkable, and she was discharged on postpartum day #2. At her six-week postpartum visit her cervical exam was remarkable for a normal appearing anterior lip; however the posterior lip was not visible and not palpable on digital exam (). Pathology was not available for the fragments of cervix that were excised and the placenta was remarkable for acute chorioamnionitis. |
pmc-6515040-1 | A 56-year-old female patient, non-smoker without major risk factors presented with a former apiectomy in frontal maxilla. Teeth #11 and 22 had fractured roots and the areas were infected. The radiological images showed severe bone loss in the frontal maxilla (#11, #12, #21 and #22). Teeth 11 and 22 already lost two-thirds of their vertical attachment. The overdenture showed some mobility and had become symptomatic, which made removal inevitable (A,B). The treatment plan consisted of the extraction of the endodontically treated teeth (#11, #22 and #12) and the removal of the prosthetics suprastructure. Following the surgical steps, an immediate implantation of two bone ring blocks (maxgraft® bonering Ø 7mm, botiss biomaterials, Zossen, Germany) and fixation of the blocks with dental implants (Ankylos Implant, Dentsply Sirona, York) was performed in regio #11 and #22 (C). The defect size was initially measured with the 7-mm trephine from the maxgraft® bonering surgical kit (botiss biomaterials, Zossen, Germany) (B). Additionally, socket preservation with gingiva graft from the tuber maxillae at #12 was performed with the goal to gain papilla through ovate pontic restoration. The site was covered with a volume-stable bovine bone substitute (cerabone®, botiss biomaterials, Zossen, Germany) and a resorbable native collagen membrane made of porcine pericardium (Jason membrane, botiss biomaterials, Zossen, Germany) (D). The post-operative check-up showed an eventless healing and healthy soft tissue 6 months after surgery (E). At that time, the re-entry was performed and healing abutments were placed in order to shape the gingiva. The graft appeared to be well integrated into the native bone (F). Six weeks later, the temporary crowns were integrated, and the first esthetic results were visible only 7.5 months after surgery (G,H). The patient was highly satisfied with the result. A radiological control at 36 months after initial surgery showed stable bone around the shoulders of the implants that indicate a mid-term esthetic outcome (I). |
pmc-6515040-2 | A 50-year-old male patient presented with multiple chronic inflammation at teeth #24, #25 and #34, endodontically treated #16 and hopeless tooth #12. The patient’s treatment options were reviewed prior to his consent to strategic extraction and implant placement. He was advised that a regenerative procedure would be necessary due to the advanced bone loss in region #12, #24–26, #35–37. His preference was an implant fixed restoration in a single-staged approach. The treatment plan was bone augmentation including sinus floor elevation in the second quadrant and implant placement in region #12, #16, #18, #24, #26, #28, #35, #37, #46, and #48. After extraction (A,B), three of the sites (#12, #24-25 and #34) were measured with the trephine from the maxgraft® bonering surgical kit (botiss biomaterials, Zossen, Germany) 7 mm in diameter. Accordingly, all of the sites were planned to be treated with 7-mm bone ring (maxgraft® bonering, botiss biomaterials, Zossen, Germany). Tooth 12 showed palatal and buccal bone loss (C,D). The site was prepared according to surgical protocol and the graft was fixated with a dental implant (Ankylos Implant, Dentsply Sirona, York). The posterior maxilla in the second quadrant was also treated with immediate implant placement (#24), a 7-mm bone ring fixated with a dental implant (#26) and external sinus floor elevation including implant placement (#28) (B) and covered with a bovine bone substitute (cerabone®, botiss biomaterials, Berlin) and a collagen membrane (Jason® membrane, botiss biomaterials, Berlin) in order to prevent resorption and soft tissue inclusion (C). The mandible was treated with a cylindrical bone block after preparing the site with a trephine and planator to create a uniform recipient site. Dental implants at #37, #46 and #47 were placed simultaneously. Eight months after surgery, the radiological image indicated well integrated bone grafts and dental implants (D). At that time, the re-entry was performed and healing abutments integrated in order to give shape to gingiva. The area showed hard and soft tissue maturation revealing what appeared to be an osseointegrated implant; both clinically and radiographically with complete regeneration of the buccal and palatal bone (D,E). Five weeks after re-entry, the soft tissues seemed healthy and final prosthesis were integrated (F). Twenty-one months after surgery, the soft tissue situation and the prosthetics looked good and the patient was satisfied with the clinical outcome (G). The 14-months post-operative radiological image showed stable implants and bone grafts (H). The 36 months image still showed similar results (I). |
pmc-6515053-1 | A 31-year-old woman with a history of left common iliac vein thrombosis presented with symptomatic lower abdominal and left groin superficial varicosities associated with itching, swelling, and discomfort. Vital signs revealed a blood pressure of 114/80 mmHg and pulse of 72 beats/minute. Physical examination confirmed the presence of large tortuous varicosities at the lower abdomen and left groin (). The patient had previously been diagnosed with left common iliac vein thrombosis during her first pregnancy, 10 years earlier, that was only managed with enoxaparin injections. Over the next few years, she developed lower abdominal and left groin varicosities that worsened significantly during her second and third pregnancies without confirmed recurrent deep vein thrombosis (DVT). The patient denied leg swelling prior to the DVT event. She also denied any history of abdominal trauma, other thromboembolic events, or family history of vascular anomalies. Computed tomography (CT) venography revealed compression of the left common iliac vein by the right common iliac artery without evidence of acute thrombosis, indicating a diagnosis of May-Thurner syndrome- (MTS-) related anatomy ().
Catheter-based venography with hemodynamic pressure measurements confirmed May-Thurner anatomy with sequelae of chronic DVT in the left iliac vein and cross-pelvic drainage via pelvic and abdominal wall varices (). Successful recanalization was performed using percutaneous transluminal angioplasty with stenting of the left common iliac vein (20 mm x 55 mm Wallstent) and left external iliac vein (14 mm x 60 mm Protege). The patient was managed with apixaban (5 mg twice daily) for three months and clopidogrel (75 mg daily) for one month that was changed to aspirin (81 mg daily) in the long term. A 3-month follow-up CT venogram indicated a patent left iliac vein stent. However, the patient continued to present with painful, though slightly improved, lower abdominal and left groin varicosities.
Given these persisting symptoms, we performed successful stab phlebectomy of the large superficial abdominal varicosity. We also treated the deeper feeding branch and groin varicosities with ultrasound-guided sclerotherapy using a sclerosing foam (two injections of 1 cc of 3% sotradecol mixed with 2 cc of room air). The procedure resulted in complete resolution of the symptomatic lower abdominal and left groin varicosities (). The patient did very well at 6- and 12-month follow-up visits while on aspirin, and a repeat CT venogram indicated a patent left iliac vein stent. |
pmc-6515062-1 | A 78-year-old woman with a past medical history significant for hypertension, intermittent asthma, prior pulmonary embolism, depression, and melanoma status after wide local excision four decades ago, was diagnosed with metastatic melanoma. Whole body positron emission tomography (PET) identified multiple metastatic lesions dispersed within the chest wall, lungs, lymph nodes, and axial skeleton. Combination immunotherapy with ipilimumab and nivolumab for four cycles, followed by nivolumab maintenance, was initiated.
Five days following the first cycle of combination immunotherapy, the patient developed diplopia and proximal muscle weakness/myalgias. Magnetic resonance imaging (MRI) was negative for metastatic disease within the brain or extraocular muscles. Given that her only other medications included amlodipine and escitalopram, it was hypothesized that these symptoms were adverse reactions to combination immunotherapy. Ipilimumab-nivolumab therapy was held and she received methylprednisolone intravenously (IV) in the clinic at a dose of 1 mg/kg body weight (75 mg).
Assessment in the hospital demonstrated abducens nerve, upward and downward gaze palsies, along with unsteady gait, and a diffuse rash. Patient had weakness and myalgias of proximal muscles bilaterally, greater in the lower extremities, and decreased vibratory sensation in the distal extremities. Vitamin B12 level was within normal limits and rapid plasma reagin (RPR) was nonreactive. Dosage of methylprednisolone was increased to 125 mg IV daily (1.5 mg/kg) due to severe clinical presentation. Routine dosing for acute myositis is methylprednisolone IV at 0.5-1.5 mg/kg; pulse therapy of 1000mg IV daily for 3 to 5 days in cases of severe myositis/lack of response or intravenous immunoglobulin (IVIG) can be initiated at 2 g/kg [].
Labs demonstrated an elevated creatine phosphokinase (CPK) of 9198 IU/L, along with a transaminitis with an aspartate aminotransferase (AST) of 683 IU/L and an alanine aminotransferase (ALT) of 315 IU/L. C-reactive protein was elevated at 39.5 mg/L. Erythrocyte sedimentation rate and thyroid stimulating hormone (TSH) were within normal limits, and hepatitis panel was negative. Myositis panel was negative for myositis-related antibodies, including Jo-1, PL-7, PL-12, EJ, OJ, SRP, Mi-2 alpha, Mi-2 beta, MDA-5, TIF-1y, and NXP-2. Due to concern for immunotherapy-related myositis, methylprednisolone therapy was continued at a dose of 125 mg IV daily. Lower extremity MRI identified moderate edema of the subcutaneous tissue, superficial fascia, and muscles consistent with myositis. The patient's troponin-I level was 8.57 ng/mL. Transthoracic echocardiogram (TTE) was within normal limits, consistent with immunotherapy-related myocarditis.
The patient had persistent proximal muscle weakness and worsening gaze palsies that were minimally responsive to steroid therapy. The dose of methylprednisolone was increased from 125 mg IV to pulse steroid dosing of 1000 mg IV daily, for a total of three days. The patient developed new bulbar symptoms including dysphagia, voice hoarseness, and ultimate respiratory distress, and inability to manage secretions. Due to bulbar symptoms refractory to high dose steroid therapy, IVIG was started at a dose of 2 g/kg IV daily for two days. A summary of the patient's treatment during her hospital stay can be seen in .
Muscle weakness mildly improved with IVIG and IV steroid therapy, but bulbar symptoms progressively worsened. IVIG therapy for severe myositis is generally loaded at 2 g/kg IV for one to two doses and then repeated every four to eight weeks. Given that the patient had already received two loading doses of IVIG with worsening clinical status, plasmapheresis was initiated. Anti-GQ1B was tested to evaluate for Miller-Fisher syndrome and was negative. Patient developed bilateral ptosis and increased respiratory effort, necessitating transfer to the intensive care unit (ICU). Anti-striational antibodies were found to be positive, with an elevated titer of 1:80, supporting a diagnosis of ICI-induced MG.
The patient's clinical condition continued to deteriorate, with progressively worsening dysphagia, increased respiratory effort, decreased ability to manage respiratory secretions, complete gaze palsy, severe ptosis, and continued proximal muscle weakness. CPK, AST, ALT, and troponins were down-trending despite worsening symptoms (). Due to increased work of breathing, there was concern that the patient would soon require intubation. The patient expressed desire to cease aggressive therapy at that time and was evaluated by the palliative care team. She was deemed to have full decision making capacity and a prognosis of days to weeks. She elected to enter hospice care, and given her increased respiratory effort and inability to manage respiratory secretions, she was discharged to inpatient hospice care. |
pmc-6515067-1 | A 32-year-old previously healthy male was admitted to our hospital with generalized abdominal pain, diarrhea, nausea, vomiting, and fever up to 39°. During assessment he was febrile without chills at 39°, BP at 135/70, pulse rate of 95 bpm, and respiratory rate at 20 breaths per minute. By his physical examination the abdomen was mildly tender to palpation with guarding in his epigastric and umbilical region.
Laboratory tests disclosed a white cell count of 14.4x1000/μL with 92% neutrophils, 3.5% lymphocytes, normal red blood cell count (5x10∧6/μL), haemoglobin (14.5g/dL), and platelets (160x1000/μL). The biochemical studies including liver, renal, and coagulation profile were normal. There were a mild hyponatremia and hypokalemia and CRP was 8.56. Cultures were obtained from blood, stool samples were obtained, and ceftriaxone and metronidazole were empirically administered.
Furthermore, no abnormalities were detected on chest and abdomen X-ray whereas an urgent abdominal ultrasound revealed thickening of the gallbladder wall, gallbladder contraction, and a minor pericholecystic fluid collection, without dilation of common bile duct or intrahepatic biliary system. In view of the clinical and ultrasonographical findings, the patient was diagnosed with acute acalculous cholecystitis and he was hospitalized initially for fluids, antibiotics, and observation.
After 36 hours of hospitalization, the patient was still febrile with fever up to 39°; however, there were obvious symptoms of toxicity. His vital points were 120bpm, BP 110/60, and 24 breaths/minute. By physical examination, the whole abdomen was contracted, with rebounding pain in the upper right upper quadrant region and Murphy's sign. Laboratory studies revealed precipitation of white blood cell count at 3.8x1000/μL with 67.4% neutrophils and 18.9% lymphocytes. Red blood cells were at 4.2x10∧6/μL, haemoglobin at 12.6g/dL, and platelets at 125.5x1000/μL (). His abdominal ultrasound showed pericholecystic fluid collection, large free fluid at Douglas's pouch, and right paracolic gutter (Figures and ). The CT-scan of the abdomen showed thickening of the gallbladder wall, pericholecystic fluid collection, without biliary dilation, a large amount of free fluid at infrahepatic area and mostly in the pelvis, and right and left parabolic gutters (Figures and ).
The patient underwent exploratory laparotomy in emergency setting, and the findings were a swollen oedematous, acalculous gallbladder with empyema, and an amount of free fluid in the peritoneal cavity. After the check of the whole abdomen, he underwent cholecystectomy. Cultures were collected from the content of gallbladder and free fluid. At the same time the first cultures isolated Salmonella at stool and in the sequel; according to antimicrobial susceptibility test, he was given ciprofloxacin and metronidazole. Pathological findings confirmed the severity of cholecystitis. The culture from content of gallbladder isolated also Salmonella. He was discharged with oral antibiotics after 8 days of his admission. |
pmc-6515074-1 | A 13-year-old boy underwent pericardial fenestration and thoracic duct ligation for pericardial and pleural effusion at 3 years of age and was diagnosed with GLA after a pleural biopsy. The patient experienced no pleural effusion before his 11th birthday. The patient had a history of cerebrospinal fluid leakage due to a skull fracture at 7 years of age. The patient was referred to our department immediately following pleural effusion when he was 11 years old. A hematological examination showed high values for D-dimer (22.2 μg/mL) and P-FDP (50.9 μg/mL). A radiograph showed pleural effusion in the right lung (). Thoracentesis revealed chylothorax mixed with blood components. Magnetic resonance imaging showed additional lesions on the lymph ducts on both sides of the inner auditory channels; computed tomography (CT) showed diffuse osteolytic changes on both sides of the femoral neck and thoracic vertebra. shows the patient's clinical course. Although the patient abstained from eating and parenteral nutrition was provided in addition to octreotide testing and pulse steroid therapy, pleural effusion worsened and became bilateral. Two or more liters were drained on days when there was a large amount of pleural effusion. We were unable to locate the site of the leakage even though we conducted a lymphogram to treat the pleural effusion and identify the leakage site. Sirolimus administration was initiated at 0.88 mg/m2/day, which proved to be an insufficient dosage. However, when the dosage was increased to 1.3 mg/m2/day after 1 month, the patient experienced an onset of disseminated intravascular coagulation (DIC) after 1 week. At that time, a blood examination showed platelet (1.4 × 104/μL), P-FDP (590 μg/mL), fibrinogen (114 mg/dL), prothrombin time rate (1.35), antithrombin (129%), and no liver dysfunction. The urine and blood cultures were negative. Viral serology was negative for cytomegalovirus, and aspergillus antigen was negative. Rheumatoid factor and antinuclear antibody were normal levels. The CT scan showed no sign of pneumonia or pyothorax. We diagnosed him with DIC using DIC score []. Although we temporarily paused the administration of sirolimus, the patient experienced an additional onset of DIC 10 days after we resumed administration. Thus, he underwent thoracoscopic removal of the hematoma (). The trough level of sirolimus during administration was 3.4–8.9 ng/mL, which does not represent abnormal elevation. Although an additional pause in the administration of sirolimus did not reduce the amount of pleural fluid, there were no additional onsets of DIC. Subsequently, there was a significant decrease in pleural fluid once Eppikajyutsuto was administered at 0.2 g/kg/day. We were able to proceed with tube thoracostomy removal 40 days after the initiation of oral administration. There was no re-accumulation of pleural fluids in the 18 months following the initiation of oral administration. |
pmc-6515135-1 | A 58-year-old obese male presented to our Emergency Department with 2-day history of progressively worsening left lower quadrant abdominal pain, urinary urgency, and 3/10 groin pain. The patient also reported dysuria. Comorbidities included hypertension and Class 1 obesity with body mass index of 32. He denied experiencing similar symptoms in the past and reported no other past medical history, no smoking history, or relevant family history.
Physical exam was significant for obvious 7 cm left inguinal hernia bulge with tenderness to palpation. On attempt to reduce the hernia, the patient reported urinary urgency. Laboratory studies and urinalysis were within normal limits. A computed tomography (CT) scan revealed left inguinal hernia containing a portion of the urinary bladder (A–C).
The patient was taken to the Operating Room the same day for planned open left inguinal hernia repair with mesh. Intra-operatively, a portion of the urinary bladder was found to be inside the inguinal hernia. The inguinal hernia sac was initially difficult to differentiate from the bladder due to its thickness (A, B). The bladder was subsequently distended with Normal Saline via the Foley catheter and a large bulge was observed in the inguinal canal, confirming the bladder herniation. The bladder was then reduced into the abdomen and the inguinal hernia and defect were fixed with a polypropylene mesh.
The patient had no intra-operative complications. On post-operative day (POD) 1, the patient appeared well with minimal groin pain. Incision was clean, dry, and with no drainage or signs of infection. Patient was able to ambulate and tolerate diet. His Foley catheter was removed and the patient was able to urinate without pain or difficulty. Patient was discharged to home on POD 2 with controlled pain, regular diet, and normalized white blood cell count. He was seen in clinic 2 weeks post-operatively free of pain and with no urinary voiding symptoms. Incision was clean, dry, and healed. |
pmc-6515138-1 | A 15-year-old female presented with a one-year history of intermittent bilateral ocular erythema, irritation, and discomfort, most severe in the right eye. She was otherwise well, with no significant past medical history or family history. Visual acuity was 6/6 in both eyes. On slit-lamp examination, giant papillae were identified bilaterally in the inferior conjunctival forniceal regions, notably larger and more widespread in the right eye (). Baseline blood tests including liver function, electrolytes, and full blood count were in normal range. The patient was initially diagnosed with allergic conjunctivitis. Whilst her ocular erythema improved with topical steroids, she experienced persistent irritation and discomfort of the right eye and represented three months later.
A biopsy of the right palpebral conjunctival lesion showed expansion of the subepithelial connective tissue by coalescent nodules of small lymphocytes. These lymphocytes had a centrocyte-like morphology; the immunophenotype is CD20+/CD10-/CD5-/CD43-. The cell markers on flow cytometry showed a monoclonal population of mature B cells with lambda light chain restriction. The morphology and immunophenotype, including immunoglobulin light chain restriction, were diagnostic of an extranodal marginal zone lymphoma of the mucosa-associated lymphoid tissue (MALT lymphoma) (). Notably, the patient's ocular examination had been atypical of the “salmon-patch” appearance that is characteristic of the condition. Lumbar puncture, bone marrow trephine, whole-body positron emission tomography scanning, and magnetic resonance imaging of the brain did not reveal any abnormalities to suggest lymphoma outside the ocular adnexal tissue. The patient was managed with a total of ten interferon alpha-2 beta injections (ten million units per dose) into the conjunctival fornix over a three-month period, evenly distributed over this time period (i.e., administered at approximately weekly intervals). Posttreatment biopsy five weeks later demonstrated reactive lymphoid hyperplasia with no clonal B cells on flow cytometry. Clinical resolution of symptoms was observed within two months of completing treatment, with no signs of recurrence up to eight years after treatment. |
pmc-6515144-1 | A 46-year-old male with a history of severe developmental delay, hydrocephalus, and seizure disorder presented to the hospital with blunt head trauma after a ground level fall. Work-up revealed bilateral acute subdural hematomas for which an external ventricular drain was placed. Despite intensive care management, the patient deteriorated to brain death. He was subsequently evaluated for organ donation. Abdominal computerized tomography (CT) scan () revealed an “elongated structure with metallic components in the upper portion of the IVC that extends into the right atrium.” There was no medical history of a prior procedure, or symptoms, to explain the incidental finding. The radiologist's interpretation and presumptive diagnosis were a retained atrial pacing wire.
He subsequently underwent procurement for organ donation after brain death. At the time of cross-clamp, the previously identified foreign body was transected when the right atrium was incised for exsanguination. During the back-table dissection, it was apparent that the foreign body had eroded into the posterior wall of the IVC, extending down the retrohepatic IVC (Figures and ). It also created a calcified reaction at the junction of the suprahepatic IVC and right atrium, adjacent to the left hepatic vein (LHV) and middle hepatic vein (MHV). We removed the foreign body () and performed a venoplasty () of the posterior wall of the IVC and of the common wall of the LHV and MVH, so that the outflow of the LHV and MHV was not compromised after transplant. We discovered that the foreign body was, most likely, a fractured CVC due to the overall appearance and interval markings.
The liver recipient was a 65-year-old woman with cirrhosis due to alcohol abuse; her Na-MELD score was 40 at the time of transplant. She underwent caval-sparing total hepatectomy and deceased donor liver transplantation via piggyback technique: the donor suprahepatic IVC was anastomosed to a common orifice of the recipient's right and middle hepatic veins. We did not alter our immunosuppressive therapy or prophylactic antibiotic regimen. Additionally, we did not initiate any anticoagulants or antiplatelet agents beyond our standard postoperative protocol. Postoperative imaging showed normal velocities and waveforms on ultrasound () and unremarkable appearance on axial CT () of the hepatic vein anastomosis. The patient otherwise had an uneventful postoperative course and has had stable allograft function with no venous outflow issues for >8 months after transplant. There were no reported complications in the other organ recipients. |
pmc-6515145-1 | Subjects from a parent-offspring trio were collected. The proband was a 23-month-old male Chinese child who presented four episodes of tonic seizures over 10 days at the age of 16 months, with normal parents. The patient had enamel hypoplasia and hypotonia of the trunk and limb muscles. His developmental quotient (DQ) and mental index (MI) scores were both less than 70. The brain MRI revealed long T1 and long T2 signal changes in the white matter adjacent to the anterior and posterior horns of both lateral ventricles, indicating dysplasia in these regions. Video-electroencephalography (EEG) monitoring showed abnormal brain waves during sleep. Increased plasma alanine aminotransferase (ALT) and aspartate aminotransferase (AST) were noted in liver function tests (ALT: 117 IU/L, normal range: 9–50 IU/L; AST: 50 IU/L, normal range: 15–40 IU/L). Plasma bilirubin was in the normal range. Elevated plasma citrulline (220.94 μmol/L, normal range: 7.00–35.00 μmol/L) and normal plasma arginine were also noted. Seven months later, his plasma ammonia level was increased to 175 μmol/L (normal range: 11–51 μmol/L) and his plasma levels of ALT (335 IU/L), AST (106 IU/L), and citrulline were higher than before. The brain MRI showed ventricular dilation and cerebral atrophy of the left hemisphere with malacia, suggesting serious irreversible brain injury. After 2 weeks of treatment with a restricted protein diet and arginine supplementation, his plasma ammonia level was decreased to 70 μmol/L. However, the patient died of a serious infection several months after hospital discharge.
Informed written consent was obtained from all participants, and the genetic analysis of the infant was performed with written consent from his parents. The study was approved by the review board of the Institute of Psychology, Chinese Academy. |
pmc-6515185-1 | A 16-year-old female with a history of OCD, MDD, and suicidal thoughts, with no past psychiatric hospitalizations, was brought to the psychiatric emergency department for two months of constant suicidal thoughts. She reported that these thoughts occurred “at night when I go to sleep and when I wake up; I am always wanting to die”. However, she felt strongly that these were not her own thoughts and that she did not actually want to commit suicide. On admission, she endorsed dysphoria, anhedonia, initial insomnia, hopelessness, worthlessness, and feelings of guilt. She also expressed “wanting to strangle the ghost out of her throat” in addition to other bizarre, intrusive suicidal and self-harming thoughts.
She endorsed a history of OCD, diagnosed in high school, and severe hypochondriasis since childhood. She has “always” had fears of being ill or contaminated and engaged in cleaning rituals. Her mother reported that she tended to phrase her obsessions in an “odd” way, for example, stating that she was “scared of breathing the air because it is heavy and dirty and full of sickness” or that she wanted to wash “slimy eels” off her hands after she touched a public handrail. This patient also had a history of depressive symptoms starting at age 7, including periodic irritability, anhedonia, difficulty in concentrating, insomnia, and worthlessness. Despite these difficulties, she was doing well in school and succeeded at playing the violin at a high level. The previous two months had been her first time having such severe, persistent suicidal thoughts.
We began treating this patient with 35 mg fluoxetine q.d. for depression and obsessive thoughts, 300 mg gabapentin q.d. for anxiety, and 150 mg quetiapine q.d. for intrusive and bizarre thoughts. In other words, this was a comprehensive treatment regimen with the goal of addressing our patient's OCD and MDD in addition to her suicidality. Her two-month hospital course was complicated by numerous episodes of heightened anxiety and suicidal thoughts, during which she was “terrified” she was going to “need to kill herself to get this ghost out”. She was worried she would act on this thought, reacting with tearfulness, feelings of hopelessness, difficulty in redirecting her emotions, and inability to concentrate on anything else. Some of her depressive symptoms such as dysphoria and insomnia decreased over her stay, but her suicidality and recurrent thoughts continued to be present. Added to the complex nature of this case was the patient's difficulty in expressing the feelings underlying her suicidal thoughts. She continuously said that these thoughts of strangling herself “came from nowhere”, and she could not pinpoint any correlation of these thoughts with her emotional state, level of distress, or feelings of hopelessness. She sometimes stated: “I want to tie something around my neck and I want to die”, or “life is so pointless”, while continuously denying to staff that she had any desire to commit suicide. The patient's family history was notable for OCD in a maternal grandfather. Of interest, the patient's identical twin sister has not had any psychiatric illnesses.
We sent this patient for neuropsychiatric testing upon recommendation from a child specialist. Neuropsychological examination found deficits in executive functioning, as assessed by a Key Search Task and the Virtual Continuous Performance Test, in addition to decreased verbal cognitive performance as assessed by the Verbal Fluency Test, Verbal Fluency Controlled Oral Word Association Test, and the Digit Span and Letter-Number sequencing subtests of the WAIS-III. The other portions of the neuropsychological exam included the Test of Variables of Attention, to assess attention; the Wechsler Memory Scale, to assess memory; the Rey-Osterrieth Complex Figure task, to assess visuospatial skills; and the Wechsler Adult Intelligence Scale, to assess general cognitive ability. The results of these other tests were all within normal limits.
The results of this testing led firstly to increased understanding by the patient's family of her mental illness; given that she had been high functioning, her parents had not understood why she was so “crazy”. The relatively objective results of this testing alleviated this pressure on our patient from her family. In addition, further treatment focused more on supporting and encouraging expression of emotion, with more time allotted for the patient to think through responses. The patient and her family were advised to increase levels of external support to help with communication and planning upon discharge.
The patient attended a psychiatric day program before returning home to be followed closely as an outpatient for the past several months. During this time, she has received standard-of-care treatment for MDD and OCD, including fluoxetine q.d. for depression, clomipramine q.d. for OCD, and cognitive behavioral therapy. Concurrently, we have worked in particular to improve her executive function and verbal abilities. The patient completes computerized working-memory training tasks daily and is being coached in mindfulness meditation, two methods that have been shown to increase executive functioning in children []. She also has engaged in auditory training exercises to improve verbal processing and memory. Currently, after more than 3 months of this additional training targeted towards neuropsychological deficits in addition to medications and therapy, this patient reports significant improvement. She has more self-efficacy and hope surrounding her mental illness and feels that she “knows what to do” if she feels that way again in the future. |
pmc-6515189-1 | A 17-year-old Japanese woman, para 0, was referred to our institution at 21 weeks and 6 days of gestation following the observation of FGR, oligohydramnios, and placental enlargement which were observed at a private clinic. Ultrasound () revealed a large placental mass with mixed high and low echogenicity. The reason underlying the enlargement of the placenta was unclear; therefore, we only measured its thickness, which was approximately 7.6 cm. We observed FGR with the following fetal biometry measures: biparietal diameter (BPD) of 31.9 mm (−6.9, standard deviation [SD]), abdominal circumference of 102.3 mm (−5.1, SD), femur length of 20.7 mm (−5.0, SD), and estimated fetal body weight of 100 g (−4.9, SD). Furthermore, severe oligohydramnios was observed, and Doppler examination revealed the absence of end-diastolic velocity (AEDV) of the umbilical artery. Dextroposition of the fetal heart was observed. Maternal serum analyses showed no TORCH syndrome. The cervical length was 8 mm, and the patient was admitted for tocolysis. However, she experienced labor pains on the following day and had a stillbirth weighing 138 g with low set ears (gender unknown) at 22 weeks of gestation, with a total blood loss of 270 g. The placenta weighed 502 g and was 8 cm in thickness. Macroscopic examination revealed that a subchorionic blood clot measuring 12 × 5 cm covered a large percentage of the placenta with well-defined margins on the fetal surface (). A large amount of blood was stored in the subchorionic space. No hematoma or infarction was observed on the maternal surface of the placenta. Microscopic examination revealed an intervillous hematoma and fibrinous deposits directly beneath the chorionic plate with adjacent compressive effects (). Based on these findings, MST was diagnosed. FGR and dextroposition of the fetal heart were observed by ultrasound, and low set ears were observed after birth. We recommended chromosomal analysis and autopsy of the fetus; however, the patient and her husband denied these. The patient had an uneventful postoperative course. |
pmc-6515553-1 | A 39-year-old woman, gravida 1, para 0, was diagnosed with invasive squamous cell carcinoma of the cervix following conization. Pathological findings showed carcinoma consistent with FIGO stage IA1 with lymphovascular invasion. She was referred to Keio University Hospital. The patient and her husband were informed of the treatment options, including AmRT and pelvic lymphadenectomy. The patient was told that the outcome of this procedure could not be guaranteed because an insufficient number of these procedures have been performed worldwide to yield reliable conclusions. The patient wished to preserve fertility, and she and her husband signed a written consent form agreeing to this treatment. Pathological findings after AmRT and pelvic lymphadenectomy showed no residual tumor and no lymph node metastasis. There was no finding of an ovarian tumor before surgery. A left ovarian cyst of 4 cm was identified during postoperative follow-up.
At 3 years and 6 months after surgery, the patient underwent IUI and then had fever and pain in her left lower abdomen 10 days later. At her first visit, her temperature was mildly elevated to 37.5°C. The patient's pregnancy was denied because a qualitative urine human chorionic gonadotropin (hCG) test was negative. A tumor with tenderness was palpated in the left adnexal area. A cystic tumor of 64x 41 mm was found by transvaginal ultrasonography (). Blood tests showed increases in white blood cell (WBC) count to 11900/μL and C-reactive protein (CRP) to 22.80 mg/dL. The patient was diagnosed with PID with ovarian cyst infection and hospitalized for treatment. Conservative treatment with antibiotics was initially used, but her symptoms did not improve. On hospital day 8, blood tests showed a further increase in WBC count to 23900/μL and CRP to 28.17 mg/dL, and pelvic CT showed that the ovarian cyst had grown to 10 cm in size ().
We decided to perform laparoscopic left ovarian cystectomy on day 8. Since the patient had a history of open surgery, adhesion was likely in the abdominal cavity. Before surgery, we asked the urologist to insert bilateral 6 Fr ureteral catheters because of possible difficulty identifying the ureters. The catheters were fixed to the thigh with tape (). The uterine manipulator was not inserted before surgery to avoid the risk of uterine perforation.
CO2 pneumoperitoneum was established at 10 mmHg. Laparoscopic left ovarian cystectomy was performed using typical trocar placement. The left ovary was swollen to 10 cm and the fluid contents were purulent (). The left adnexa, posterior uterine wall, and retroperitoneum were firmly adhered (). The bilateral fallopian tubes were firmly adhered to the surrounding tissue and were unable to be identified. During surgery, by moving the catheter manually back and forth from outside the body, we were able to identify the ureters visually (). The 7 cm uterine manipulator was inserted during surgery under a laparoscopic view. The left ovarian cyst was excised, leaving the normal part of the ovary (). The operation time was 2 h and 58 min, and blood loss was 550 mL. No complications occurred during or after surgery. After the operation, symptoms improved rapidly and the patient was discharged 8 days after surgery. The excised specimen was pathologically an endometriotic cyst. A bacterial culture of the cyst fluid was positive for Prevotella bivia, Prevotella species, and Finegoldia magna. |
pmc-6515610-1 | A 21-year-old Caucasian female patient presented to our consultation with CNO of her left clavicle. The associated pain was recurrent, progressive and eventually led to work stoppage as a secretary. Symptoms started at the age of nine, with initial plain radiographs showing fusiform sclerotic bone remodeling of the medial third of the left clavicle. There was no further relevant past medical history. Percutaneous bone biopsy at that time revealed fragments of immature woven bone, with no signs of malignancy. Histopathological and microbiological analyses excluded etiologies such as infection, neoplasia, vascular or metabolic disorders, and the diagnosis of CNO was retained. She was thus treated conservatively with NSAID.
Over the years, she started complaining of paresthesia, weakness and pain radiating to her left arm on abduction or elevation of her left shoulder. Clavicle diameter reached 6 cm on computed tomography. Lateral progression of osteosclerosis was noted, with involvement of the acromioclavicular joint and compression of the brachial plexus and subclavian vessels (Fig. ). Malignant transformation was excluded by a further surgical bone biopsy at the age of 20, but wound dehiscence and chronic cutaneous fistula developed a few months later.
At the time the patient (aged 21) was referred to our institution, she had a 5/10 pain score at rest using the visual analogue scale (VAS). The shoulder ROM was limited to 90° of flexion and elevation, while internal and external rotation were preserved. The Constant-Murley shoulder outcome score was at 35/100 (this score evaluates pain, activities of daily living, strength and shoulder ROM []). We then proposed and performed a two-stage reconstruction of the left clavicle (Fig. ). Following claviclectomy, the defect was filled with a custom-made antibiotics-loaded cement spacer, allowing the formation of an induced membrane according to the Masquelet technique, while total clavicle reconstruction using a free vascularized peroneal graft was performed 8 weeks after the index procedure (Fig. ).
For surgical reconstruction, a 3D-printed model based on the healthy contralateral right clavicle was used to conform the new left clavicle. After harvest, the free fibula was positioned in the Masquelet cavity (Fig. ). All four medial and lateral anchorings of the clavicle were reconstructed. Hamstring tendons were passed through bone tunnels in the sterno- and acromioclavicular joints. Tension in anchorings was low to allow for free shoulder elevation and antepulsion up to 90°. Stabilization was reinforced with suture anchors placed at the base of the coracoid and on the antero-superior aspect of the first rib (Figs. and ). Finally, the vascular bundle was anastomotized to the thoracoacromial artery and a branch of the external jugular vein (Fig. ).
Perifistular cultures revealed usual skin pathogens. Macroscopically, we observed no signs of suppurative infection or sequestration. Histologically, there were intratrabecular spaces filled with loose fibrovascular tissue containing inflammatory cells (mainly lymphocytes, plasma cells, macrophages and a few polymorphonuclear cells). Periodic acid-Schiff, Grocott, Gram and Ziehl-Neelsen colorations were all negative. We found no evidence of organisms, eosinophils, granulomatous foci, abscess formations, sequestrae, and signs of malignancy or Paget’s disease. Clinical presentation, imaging findings and histopathological analyses led to the final diagnosis of CNO.
Postoperative evolution was favorable with eventless wound healing. The left shoulder was immobilized for 6 weeks, then progressive active and passive motion were started. Follow-up consultation at 23 months showed satisfactory cosmetic and functional outcomes. Left shoulder ROM returned to the level of preoperative assessment 3 months after surgical reconstruction. While pain VAS was 2/10 at 3 months, it was 0/10 at last follow-up (23 months) without pain medication. The evolution of the Constant-Murley shoulder score was satisfactory with an improvement to 68 points at 2 years. The long-term evolution met the patient’s satisfaction, which was evaluated at 8 on a 10-point rating scale. |
pmc-6515619-1 | A 52-year-old Italian man sought care at the Emergency Department (ED) of Luigi Sacco Hospital in Milan, Italy on 14 December, 2017, complaining of a quartan pattern of fever that started 1 week before together with arthralgia and myalgia. He reported frequent trips to sub-Saharan Africa, the last one to Mozambique and several previous malaria attacks treated by himself using quinine. He reported to have not taken anti-malarial chemoprophylaxis. A chest X-ray was negative and laboratory examinations were unremarkable except for an increase of C reactive protein (50.9 mg/L) and mild thrombocytopaenia (153,000/µL). A blood smear was negative for malaria parasites as well as a rapid diagnostic test (RDT), but species-specific PCR turned positive for P. malariae. He was treated with a standard regimen of oral chloroquine phosphate (1 g per os initially, 500 mg 6 h after the first dose, and then 500 mg once a day on the 2nd and 3rd days of therapy). Subsequently he was in good health until the end of April when fever recurred spiking to 40 °C associated with severe headache. On 4 May, 2018 he presented to the ED of another hospital where a blood smear was positive for trophozoites of Plasmodium spp. He was transferred to the ED of L. Sacco hospital where a new blood smear showed scanty trophozoites of P. malariae; RDT was negative and species-specific PCR confirmed the diagnosis of P. malariae. Clinical examination was remarkable for the presence of herpes labialis, but otherwise negative. A chest X-ray was negative and blood examinations showed increase C-reactive protein (201 mg/L) mild anaemia (Hb 12.2 g/dL, Ht 35%), leukopaenia (WBC 3200/μL) and thrombocytopaenia (45,000/μL). In the period between the two P. malariae episodes he admitted only a short stay in northern Africa (Libya) without any other trip to sub-Saharan Africa. He was admitted to the Infectious Diseases Ward and treated with a 3-day course of dihydroartemisinin–piperaquine (320/40 mg) 4 tablet/day for 3 days. He was discharged on 8 May, 2018 with negative blood smear and PCR for malaria. On follow-up he had normalization of blood examinations and up to January 2019 no more recurrences of malaria. |
pmc-6515634-1 | The patient was a 76-year-old woman with a history of left renal thromboembolism, hypertension, atrial fibrillation, hyperthyroidism, stroke, and AAA. Two years before admission, she had undergone EVAR with a stent graft for infrarenal aortic aneurysm, which led to right ostial RAO. Her left kidney was already atrophic due to previous renal thromboembolism, causing her to develop dialysis-dependent renal failure immediately after EVAR. Twenty-two days after EVAR, she underwent successful PTRA and stenting, and hemodialysis was discontinued (Fig. a). However, 2 years after EVAR, she developed acute onset of leg edema, anuria, and hypertension for 2 days, and thus went to the hospital. She was found to have significant worsening of renal function, with a serum creatinine level of 2.4 mg/dL, which had increased from a recent baseline level of 1.0 mg/dL. Acute kidney injury (AKI) due to renal artery in-stent restenosis was suspected, and she was admitted to the hospital. On day 2 of hospitalization, renal artery angiography demonstrated that the right renal artery could not be visualized, thereby confirming the diagnosis of AKI due to right renal artery in-stent occlusion. On the same day, PTRA for the right renal artery was attempted; however, it was unsuccessful as the guide wire could not pass through the ostium of the renal artery (Fig. b). Given that her renal function did not improve and anuria persisted, hemodialysis was initiated on the same day. She was transferred to our hospital for preparation of regular hemodialysis on day 9 of hospitalization.
On admission, she had a blood pressure of 202/109 mmHg, heart rate of 76 beats/min, and respiratory rate of 16 breaths/min. During examination, no extremity edema was noted, and no bruit was detected on abdominal auscultation. Her laboratory results at the time of admission were as follows: serum creatinine level, 9.5 mg/dL; estimated glomerular filtration rate (eGFR), 3.5 mL/min/1.73 m2; serum albumin level, 3.2 g/dL; serum potassium level, 3.8 mEq/L; serum calcium level, 8.6 mg/dL; serum phosphate level, 4.7 mg/dL; and hemoglobin level, 10.1 g/dL.
Anuric AKI persisted after she was transferred to our hospital. In addition to RAO, we suspected contrast-induced nephropathy or cholesterol emboli as a differential diagnosis. However, these were unlikely as she developed AKI before contrast medium was administered and before the intravascular catheter procedure was performed; moreover, there were no signs of peripheral atheroembolism. Her high plasma renin activity (10.7 ng/mL/h, with a plasma aldosterone level of 140 pg/mL), which was tested to determine the cause of hypertension, raised the suspicion that she had a minimally but significantly perfused right kidney. Doppler ultrasonography revealed a renal interlobular artery flow inside the right kidney, indicating that the perfusion of the kidney was sustained, at least at a low level. Renal mercaptoacetyltriglycine (MAG-3) scintigraphy revealed that the right kidney had stained slowly and MAG-3 had not washed out even at 66 min post-injection. The absence of MAG-3 washout meant that the function of the kidney decreased severely, which did not rule out contrast induced AKI, but at least indicated that while renal perfusion was sustained, it was not sufficient to sustain the GFR (Fig. a). Although we initially planned to create an arteriovenous fistula for vascular access for permanent hemodialysis, the results of these renal imaging studies led us to reconsider that the AKI may be reversible if the renal artery was revascularized. However, a second attempt of PTRA was considered challenging, given that the renal artery was almost completely occluded. With no prior experience performing hepatorenal bypass, a transplant surgeon on our team considered kidney AutoTx to be a feasible alternative; therefore, kidney AutoTx was performed on day 25 of hospitalization. The renal artery and vein were anastomosed to the external iliac artery and vein, respectively. Cold and warm ischemia time of the right kidney were 84 and 7 min, respectively. After declamping the external iliac artery, the kidney perfused well, except for the upper one-fifth portion, which appeared to be perfused by an upper polar renal artery. Hence, we decided to anastomose the upper polar renal artery to the inferior epigastric artery; this successfully perfused the upper kidney region. Intraoperatively, the patient started passing urine. For the first 24 h postoperatively, her urine output was 2.2 L, and hemodialysis was discontinued (Fig. ). Her serum creatinine level decreased to 1.02 mg/dL by postoperative day 22, which was almost equivalent to her baseline creatinine level. Her hypertension improved, and the dose of nifedipine was successfully reduced. Her 0-h kidney biopsy during the surgery showed only mild tubular injury and no tubular necrosis. Of 24 glomeruli, 11 showed global glomerular sclerosis, and the remaining 13 exhibited no acute changes. Mild arteriosclerosis with only one cholesterol embolism was observed. Thus, no histological changes were noted that could account for prolonged ischemia leading to anuric AKI (Fig. ). The patient was discharged on day 42 of hospitalization, and her kidney function remained stable for 2 years, with a creatinine level of 1.03 mg/dL. Four months after surgery, a repeat renal MAG-3 scintigraphy showed that the transplanted right kidney at the right iliac fossa stained smoothly, and MAG-3 washed out 15 min after injection, indicating that renal perfusion and glomerular filtration has recovered (Fig. b). |
pmc-6515649-1 | A 66-year-old Sri Lankan woman who had been followed in a dermatology unit for 34 years for diffuse systemic sclerosis presented to our institution with an acute exacerbation of the skin disease. She was treated with corticosteroids and cyclophosphamide pulses and subsequently with mycophenolate mofetil for the skin condition. She did not have any other past medical or family history of systemic diseases, chronic infections, malignancies, or genetic diseases. She was a housewife, was unemployed, and was not exposed to any indoor or outdoor toxins, chemicals, or radiation. She was a nonsmoker and nonalcoholic.
On examination, she had widespread thickening of the skin predominantly involving the trunk and proximal extremities (Fig. ). She did not have sclerodactyly, but she had deformities in keeping with osteoarthritis (Fig. ). She denied cold-induced episodic acral bluish discoloration suggestive of Raynaud’s phenomenon. She was not febrile, and the result of her general examination was normal without pallor, cyanosis, clubbing, lymphadenopathy, or bilateral ankle edema. Her respiratory and cardiovascular examination results were normal with a heart rate of 82 beats per minute and a blood pressure of 130/80 mmHg. The result of her neurological examination was normal with normal funduscopy without any cranial neuropathy or peripheral neuropathy. Repeated echocardiography did not reveal any evidence of pulmonary hypertension. Upper gastroduodenoscopy did not show reflux disease. Radiographically, there was no evidence of interstitial lung disease. The patient’s autoimmune antibody profile (antinuclear antibody, anti-double-stranded DNA, perinuclear antineutrophil cytoplasmic antibodies, cytoplasmic antineutrophil cytoplasmic antibodies, anti-Smith antibody, anti RO and anti-LA, antitopoisomerase antibody, anticentromere antibody, and complements) was persistently negative, and her full blood count, urine full report, and renal and liver function were normal (Table ). Absence of Raynaud’s phenomenon; sclerodactyly; characteristic lung, gastrointestinal, and cardiac involvement of systemic sclerosis; and repeatedly negative antinuclear antibody test results lead us to reevaluate the patient for the possibility of scleredema.
Skin biopsies from four body sites showed normal epidermis and thickened reticular dermis with swollen collagen bundles separated from one another by clear spaces, resulting in fenestration. The skin appendages were not atrophied or bound down. Alcian blue staining showed interstitial mucin deposition suggestive of scleredema. Serum protein electrophoresis demonstrated an abnormal monoclonal band in the gamma region with a paraprotein level of 8.9 g/dl. Immunofixation showed an abnormal band in the gamma region consisting of IgA and κ. Bone marrow biopsy revealed abnormal monoclonal plasma cells (15%) with multinuclearity. There was no evidence of end organ damage with normal calcium, renal function, and full blood count, and whole-body magnetic resonance imaging did not reveal any evidence of bone involvement (Fig. ). The patient’s diagnosis was revised as scleredema type 2 associated with IgA-κ smoldering myeloma. She was commenced on intravenous immunoglobulin (IVIG) monthly (1 g/kg for 2 days per month), and a hemato-oncologist started intravenous bortezomib cycles (1.7 g on day 1, day 8, day 22, and day 29). Currently, she was receiving 6 months of IVIG and four cycles of intravenous bortezomib, and significant improvement of the skin was observed. |
pmc-6516587-1 | A 60-year-old gentleman with past medical history of liver transplant five years ago presented to the hospital with acute onset of right-sided knee pain. For his immunosuppressive regimen, he took 2 mg/day of tacrolimus. His complete medication history was reviewed and no significant drug-drug interactions were found. His social history was negative for excessive alcohol use and high-protein diet. His physical examination was significant for right knee warmth, swelling, and erythema with tenderness upon palpation.
Labs indicated normal white blood cell count, normal creatinine at 0.81 mg/dl, tacrolimus at 9.3 ng/ml, uric acid at 6.1 mg/dl, and elevated C-reactive protein at 18.1 mg/L. Synovial fluid analysis showed 27,000 nucleated cells with differential of >90% neutrophils and 1+ monosodium urate crystals (Table ). Fluid cultures were negative and ruled out septic arthritis. This patient was diagnosed with acute gouty arthritis, and the patient was administered colchicine for three days. His tacrolimus dosage was decreased from 2 mg/day to 1 mg/day. With treatment, the patient’s symptoms resolved, and he was continued on the adjusted dose of tacrolimus with outpatient follow-up. |
pmc-6516614-1 | Presenting symptoms and workup
A 22-year-old male presented with low back pain for five years with a recent onset of severe radicular symptoms. His past medical history was significant for Hodgkin's lymphoma diagnosed in December 2014 that was treated with chemotherapy and radiation to the mediastinum and right hip, as well as osteoblastoma of the right hip treated with surgery and radiofrequency ablation in July 2010. The pain was described as originating from the low back and radiating around his right hip and laterally down the right leg to mid-calf with a shooting-like quality. There was no associated numbness nor bowel or bladder incontinence. He has a known right L5 laminar sclerotic lesion measuring 11 x 10 mm causing neuroforaminal narrowing and increasing in size despite previous treatment with stereotactic radiosurgery and radiofrequency ablation in October 2016 (Figure ). This lesion encroached near the pars interarticularis and was metabolically active on positron emission tomography-computed tomography (PET-CT) imaging (Figure ). He reported worsening pain that was refractory to physical therapy, NSAIDs, aspirin, muscle relaxants, lidocaine patches, and radiation therapy. The patient is allergic to sulfa drugs and has a non-contributory social history. Pertinent family history involved his father who died from non-Hodgkin’s lymphoma at the age of 52. On physical examination, the patient had full strength in all extremities including bilateral iliopsoas, gluteals, quadriceps, hamstrings, tibialis anterior, extensor hallucis longus, and gastrocnemius. All reflexes were within normal limits and there were no signs of upper motor neuron disease or muscle atrophy.
Surgical approach
The patient was positioned prone on a Jackson table with padding under all pressure points. A midline lumbar incision was made and a right-sided dissection through the lumbosacral fascia was performed in a subperiosteal fashion. An X-ray confirmed the appropriate level. The right L5 lamina was expanded and raised from the tumor. A partial laminectomy was performed using a high-speed drill to resect the abnormality in an en-bloc fashion, while being careful not to violate the pars interarticularis. The lesion did not involve the inner cortex of the bone. Pieces of Surgicel were used to create a barrier between the cement and the spinal canal. Lamina reconstruction was achieved with bone cement augmentation for preservation of vertebral column strength and prevention of potential fracture of the pars interarticularis.
Pathology
The pathology was consistent with osteoid osteoma with marrow edema and areas of bone remodeling. No granulomas or evidence of Hodgkin’s disease was identified. Microscopic findings include bony trabeculae associated with prominent rimming and hypercellular fibroblastic stroma (Figure ). No nuclear atypia, necrosis or appreciable mitotic activity was observed.
Postoperative course
The patient remains neurologically intact with significantly improved radicular symptoms and low back pain. He has decreased his pain medication need and reports improved ambulation 1.5 weeks following surgery. |
pmc-6516615-1 | A 37-year-old male with a history of severe IBD, on mesalamine and infliximab, and non-ischemic stress-induced cardiomyopathy with a recovered ejection fraction of 50%-55% two years prior presented to the emergency department with complaints of moderate to severe, worsening, constant substernal pleuritic chest pain for three days. He endorsed a dry cough and low-grade fevers. He denied orthopnea, paroxysmal nocturnal dyspnea, weight gain, leg swelling, palpitations, or pre-syncope. He also reported three to four dark, bloody bowel movements a day accompanied by abdominal pain and tenesmus for two weeks. These symptoms were attributed to Crohn's flare-up, prompting the initiation of prednisone 40 mg daily for one week followed by a taper with an increase in the dose of mesalamine from 1200 mg to 1600 mg three times daily. He experienced minimal improvement in his gastrointestinal symptoms. The patient denied the use of tobacco, alcohol, or other illicit drugs. Of note, he reported feeling a similar chest pain during his flare-up two years prior.
At the time of arrival, the patient was febrile to 100.60 F, with other vitals unremarkable. Mucous membranes were pale and dry. Lungs were clear to auscultation. The patient had a hyperdynamic precordium, loud S1 and S2, and grade II pan-systolic murmur heard at the apex with radiation to the axilla. He had tenderness to touch in the right lower quadrant with associated guarding and rigidity, but no rebound tenderness. He had regular peripheral pulses with well-perfused extremities. Electrocardiogram (EKG) revealed ST-segment elevation in leads I and aVL (Figure ). Laboratory work revealed leukocytosis of 26.2 (4.0-10.5 k/uL), chronic stable anemia of 9.5 (12.5-16 g/dL), and unremarkable basic metabolic panel (BMP). Troponin I was elevated to 1.82 (<0.4 ng/mL), ESR 121 (0-22 mm), and CRP 180.1 (<3.0 mg/L). A computed tomography (CT) scan of the abdomen showed distension and wall thickening of the jejunum and proximal sigmoid colon consistent with colitis in the setting of Crohn’s flare-up.
ST-segment elevation along with the troponin leak was suspicious for acute coronary syndrome. (ACS). However, two years prior, the patient had an angiogram showing normal coronary arteries. Given the absence of significant atherosclerotic coronary artery disease, his presentation raised suspicion of acute myopericarditis in the setting of Crohn's disease flare-up. The patient was admitted in the hospital and started on colchicine with an increase in the dose of prednisone to 40 mg daily. Mesalamine was discontinued. Cardiac MRI was done and resulted in early and late gadolinium enhancement along with metrics for myocardial edema consistent with myocarditis (Figure ).
The patient’s symptoms improved with steroids and colchicine. His troponin peaked at 7.21 ng/mL and subsequently trended down. He was eventually discharged on colchicine and a slow steroid taper for 10 days. He followed up with the clinic and was symptom-free. |
pmc-6516617-1 | A 62-year-old male from Tennessee with a past medical history of janus kinase 2 (JAK2) positive essential thrombocytosis diagnosed in 2014, hypertension, hyperlipidemia, and major depressive disorder presented to the emergency department (ED) with acute encephalopathy. Due to initial encephalopathy, information regarding medical history was obtained by review of previous hospital documentation obtained from his hometown. The patient was previously taking anagrelide 0.5 mg twice a day (BID) then hydroxyurea 500 mg BID for myeloproliferative disorder, however, he discontinued the medications in 2015 due to depression.
His medical history is notable for a hospitalization four months ago where he presented with fatigue, weight loss, and cough, and was found to have an enlarged spleen. During that time, a computerized tomography (CT) scan of the chest, abdomen and pelvis was performed which noted 4 x 4 x 2.3 cm mass in the right adrenal gland, and splenomegaly. Further workup at that time revealed multiple brain lesions on brain imaging, with an unclear source. The patient also had a lumbar puncture (LP) with normal cerebrospinal fluid (CSF) results, negative human immunodeficiency virus (HIV), negative acid-fast stain and toxoplasmosis. Bone marrow biopsy in the past revealed findings consistent with a myeloproliferative disorder. The patient was scheduled to undergo adrenal biopsy, however, he declined this and decided to leave the hospital. Additionally, the patient saw a neurologist one month ago for persistent neck pain for the past seven months, associated with numbness and tingling in his right hand, which had subsequently progressed to his right forearm and upper arm. Magnetic resonance imaging (MRI) of the brain showed numerous supratentorial and infratentorial ring enhancing lesions. The differential diagnosis at that time included metastatic disease, and atypical infection such as toxoplasmosis. A lumbar puncture was done, however, official results were not available except for the cytology which was showing no evidence of malignancy. The week prior to this hospital admission, the patient had a transient episode of difficulty speaking. He called his doctor and was advised to go to the ED for evaluation, however, he declined to do so.
The patient presented to our hospital this time with altered mental status for the past 24 hours. He was unable to give any history at the time of presentation, although a friend at bedside was able to recall the patient complaining of fevers, weakness, and a headache the day before. Upon arrival to the ED, he was tachycardic, febrile, and saturating well on room air. Initial labs revealed an elevated creatinine of 1.5 mg/dL and an elevated lactic acid of 2.7 mmol/L. The patient was started on broad spectrum antibiotics with cefepime and levaquin. CT of the brain without contrast showed multiple hypointense lesions with a hemorrhagic component (Figure ).
An MRI of the brain with contrast was done which revealed numerous masses throughout the brain parenchyma involving all lobes (Figures -).
MRI of the cervical, thoracic and lumbar region also revealed numerous ring enhancing lesions (Figures -).
The patient was started on Decadron and Keppra at this time. His antibiotic regimen was transitioned to liposomal amphotericin and meropenem. A CT chest, abdomen and pelvis was then ordered to search for any potential primary tumor. Results were significant for bilateral adrenal masses, 4.1 x 3 cm on the right, and 1.6 cm on the left, and splenomegaly (Figure ).
Due to the patient’s poor mental status a continuous video electroencephalogram (EEG) was performed which revealed bilateral slowing consistent with encephalopathy and no epileptiform discharges or seizures. It was decided to next proceed with a lumbar puncture. LP revealed CSF glucose 28 mg/dL, protein 138 mg/dL, and white blood cell count (WBC) 43. Cryptococcal antigen is negative. Flow cytometry of the cerebrospinal fluid revealed no evidence of a B-cell or T-cell lymphoma. Antinuclear antibody (ANA) and hepatitis panel were also negative.
Due to LP findings consistent with low glucose levels the decision was made to start the patient on empiric antibiotics targeting fungal species. The patient was also started on high-dose steroids and maintained on this therapy with appropriate seizure prophylaxis.
The patient underwent right frontal stereotactic needle biopsy of right frontal lobe ring-enhancing lesion with the use of MRI-guided frameless stereotactic navigation. Brain biopsy pathology showed reactive astrocytosis, small narrow neck budding fungi consistent with Histoplasma species with secondary necrotizing vasculitis. Acid-fast bacilli stain performed was negative. Follow-up serology testing showed Histoplasma yeast 1:16, and Histoplasma mycelia 1:8. Gonorrhea and chlamydia polymerase chain reaction (PCR) were negative. Testing for rapid plasma reagin (RPR) and fluorescent treponemal antibody absorption (FTA-ABS), methicillin-resistant staphylococcus aureus (MRSA), and HIV were negative. CD4 count was 100.
His antibiotic regimen was switched to meropenem, amphotericin and itraconazole. However, the patient had deterioration in his neurological status becoming obtunded. He became minimally responsive to verbal stimuli and the family decided to proceed with hospice due to worsening clinical course and poor prognosis. |
pmc-6516622-1 | A three-year-old female presented to our emergency department with a three-week history of productive cough, rhinorrhea, non-bloody non-bilious emesis, and intermittent fevers with a maximum temperature of 102 °F. The child was treated for pneumonia on two occasions in the past six months. There was also a history of recurrent ear infections and she was diagnosed to have moderate persistent asthma a year ago. Her birth history was uncomplicated and there was no history of any structural heart disease or any other congenital defects. The child was up to date with vaccines including four doses of pneumococcal conjugate vaccine (PCV)13. There was no family history of recurrent infections, immunodeficiency, consanguinity, or cardiac problems.
On detailed physical exam, subcostal retractions were noted with crackles and decreased air entry on the right side. In light of the respiratory distress, a chest radiograph was obtained and demonstrated an opacification in the right lower lobe, and hence she was admitted with a diagnosis of pneumonia. The following morning, a new soft 2/6 systolic murmur was auscultated over the cardiac apex. Cardiac echocardiography was performed, which revealed 3-mm vegetation on the anterior mitral valve leaflet. Initial laboratory evaluation showed a white count of 23 X103/cu.mm3, with 83.4% neutrophils. Her C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR) were 131.17 mg/dL and 35 mm/hr, respectively. Human immunodeficiency virus (HIV) test was resulted to be negative. The primary blood culture grew Streptococcus pneumoniae, serotype 3N sensitive to penicillin G and ceftriaxone. Based on the clinical finding of a new murmur with cardiac vegetations on the echocardiogram and the blood culture report, the patient was diagnosed to have IE and was treated with IV ceftriaxone for four weeks. CBC and CRP were trended throughout her hospital stay until her leukocytosis resolved and CRP normalized. Follow-up echocardiogram one month later showed resolution of vegetation and the murmur.
Final diagnosis
The final diagnosis was IE due to S. pneumoniae, serotype 3N. |
pmc-6516623-1 | We present the case of a 56-year-old postmenopausal woman with a past medical history significant for asthma and anxiety disorder who presented with a one-month history of progressive myalgia, dizziness, exertional dyspnea, cough, and fatigue preceded by a prodrome of mild fever and upper respiratory tract infection symptoms. Apart from a recent travel to Peru and a hiking trip in New Hampshire, she denied any other exposures. She does not have any significant family history. Her husband was recently treated for Lyme disease. At the time of admission, her blood work revealed a white blood cell count of 12,000 cells/µL, hemoglobin of 9 g/dL, and platelet count of 22,000 cells/µL. She was started on doxycycline for presumed tick-borne illness, but on treatment, her symptoms continued to worsen. She was evaluated with iron studies, vitamin B12, folate levels, hemolytic panel, liver function test, and serological testing of tick-borne illnesses, all of which yielded unremarkable results. A peripheral smear showed normocytic normochromic red blood cells (RBC). She required packed RBC transfusion for symptomatic anemia. Because of worsening anemia and thrombocytopenia, bone marrow aspiration and biopsy were performed which showed metastatic lobular carcinoma of the breast (Figure ).
Immunostains showed that the tumor cells were positive for cytokeratin 7 (CK7), cytokeratin AE1/AE3, estrogen receptor (ER) (about 80%, strong) and negative for cytokeratin 20 (CK20), E-cadherin, thyroid transcription factor 1 (TTF-1), progesterone receptor (PR), and human epidermal growth factor receptor 2 (Her2); this raised suspicion of breast cancer. Breast examination was conducted at that time, and around 2 cm x 2 cm mass was palpated in left breast. A mammogram and breast ultrasound were also obtained, revealing a 1.9 cm x 1.9 cm x 1.3 cm primarily hyperechoic heterogeneous mass with multiple regions of antiparallel hypoechoic spiculated densities with posterior shadowing. To rule out other metastasis, positron emission tomography/computed tomography (PET/CT) scans were obtained (Figure ) showing focal medial wall thickening in the cecum. We performed a colonoscopy with biopsy which showed metastatic breast cancer. Therefore, our patient was diagnosed with stage IV breast cancer with ER+, PR-, Her2‑.
Our patient was then started on letrozole and palbociclib. Soon after starting the therapy, she developed immune thrombocytopenia and autoimmune hemolytic anemia. The results of her direct antiglobulin test were positive for immunoglobulin G. Therefore, she received steroid therapy which was tapered slowly over two to three months, and her palbociclib and letrozole therapy was discontinued. The patient was started on chemotherapy with capecitabine. She had a good response to this therapy with counts slowly returning to reference range over the next four to six months. Her follow-up scans showed no new metastatic disease, and we noted significant shrinkage of the known breast mass. She tolerated this therapy very well. |
pmc-6516625-1 | A 36-year-old female was discovered to have an astrocytoma after she presented with new onset seizures. She was started on levetiracetam to control the seizures. Seven weeks later, she presented with break-through seizures while the serum levetiracetam level was therapeutic. Lamotrigine was then added to her medications. Four weeks later, she presented with a rash, right upper quadrant abdominal pain, and fever for four days. She denied any nausea, vomiting, diarrhea, or joint pains. Her medical history was significant for allergies to nuts and pollen. The family history was not significant. She denied having traveled out of the city in the last 18 months. She was a stay-at-home mother of one child.
On physical examination, she was in mild distress due to abdominal pain, febrile to 38 degree Celsius, pulse of 110 per minute, respiratory rate of 18 breaths per min, blood pressure of 124/77 mm Hg in the right upper limb in the supine position, and oxygen saturation of 98% on room air. A reticulated macular erythematous rash was noted on the upper extremities (Figure ) and trunk (Figure ). The head and neck exam did not demonstrate any localized swelling, lymphadenopathy, or icterus. There was no oral ulceration or conjunctival injection. Cardiac and lung exam were within normal limits. The abdominal exam revealed right upper quadrant tenderness with Murphy’s sign. Initial lab data demonstrated an eosinophil count of 4 x 10^5/ml, elevated white cell count, alanine aminotransferase (ALT) of 1231 units/L, and aspartate aminotransferase (AST) of 1026 units/L. Alkaline phosphatase was 362 units/L. Lamotrigine was stopped as DRESS was considered. Over the next 48 hours. there was no improvement, against expectations. An ultrasound of the abdomen followed by magnetic resonance cholangiopancreatography confirmed the presence of acalculous cholecystitis. Antibiotics were administered for cholecystitis. Two days later, her abdominal pain subsided but she remained febrile (T-max of 39.3 Deg C). By now, the reticulated erythema spread to her face and lower extremities (Figure ). Her face became edematous, and she developed enlarged salivary glands and lower lip edema (Figure ). Her pharynx was noted to be erythematous and her tonsils were enlarged and covered with exudates. Cervical and submandibular lymphadenopathy developed as well. At this time, an alternate diagnosis was entertained while she did not improve over the next one week. She was immune to hepatitis A and B. Antibody to hepatitis C was negative. The antinuclear antibody, anti-smooth muscle antibody, antimitochondrial antibody, and liver-kidney microsomal assay were negative. Serum ceruloplasmin was within normal limits. Serum toxicology showed a salicylate and acetaminophen level within normal limits. Urine toxicology was negative for substances of abuse. Serum titers for rubella, rubeola, mumps, Epstein Barr virus, cytomegalovirus, varicella zoster, and human herpesvirus-6 were negative. Anti-streptococcal titers, throat culture for Streptococcus, and diphtheria culture were sent as well and reported no growth or negative growth. The cell count peripheral smear did not demonstrate mononucleosis or atypical lymphocytosis. A chest radiograph demonstrated a newly developed right-sided pleural effusion without evidence of consolidation. A skin biopsy was done, which confirmed perivascular dermatitis such as that seen in drug hypersensitivity (Figures -).
About 10 days after presentation, the levetiracetam was discontinued because of the lack of improvement in symptoms with the cessation of lamotrigine. With that, she began to improve gradually with the disappearance of the skin rash over the next few days, resolution of the facial and the lymph node swelling, as well as a return of transaminases to the baseline over the next few weeks. |
pmc-6516626-1 | A male patient aged 49 years with no previous medical history presented with neck pain from a foreign object. The patient was accidentally struck by a projectile from a nail gun on a construction site. The patient complained of pain along the left side of the neck and inability to swallow. On initial evaluation, patient was neurologically intact with absent Horner's syndrome. There were no signs of active bleeding or hemodynamic instability. The head of the nail was visible behind and above the angle of the mandible at the level of the skin - neck zone 3 (Figure , Table )
The point of the nail was not visible in the oral cavity but was palpable along the oral mucosa along the left side of the oropharynx. The patient was intubated and sedated for airway protection without complications in an outlying emergency room (ER) and triaged to the local Level One trauma center for further management. Upon arrival to our trauma center, a computed tomography (CT) angiography of the neck was obtained which demonstrated the nail traversing in very close proximity to the left internal carotid artery close to the skull base with preserved flow proximal and distal to the nail. There was no active contrast extravasation or definitive presence of a pseudoaneurysm although the evaluation was limited by the beam-hardening artifact from the nail (Figure ).
The patient was emergently transported to the interventional radiology suite to determine the status of the left carotid artery and possible endovascular repair. Left common carotid angiography in the neck demonstrated preserved flow through the left internal carotid artery but significant vessel narrowing at the level of the nail (Figure ).
Further investigation with three-dimensional (3D) rotational angiography demonstrated small areas of contrast stagnation proximal and distal to the nail with a small dissection flap proximal to the nail, consistent with at least a partial vessel injury (Figure ).
The intracranial left anterior circulation imaged normally without abnormal vessel dropout. Upon discussion among trauma surgery, oral and maxillofacial surgery and neurointerventional teams, the decision was made to proceed with endovascular repair in conjunction with nail removal. The patient was given 600 mg of clopidogrel and 325 mg of aspirin via orogastric tube and was heparinized to an activated clotting time (ACT) greater than 250. The short 6F sheath was exchanged for 6F Terumo Destination sheath (Terumo Medical, Somerset, New Jersey, USA) and positioned in the distal left common carotid artery. The injury site was crossed with a Synchro-2 0.014 microwire (Stryker, Fremont, California, USA) under the fluoroscopic roadmap. A 5 x 25 mm Gore Viabahn covered stent (W.L. Gore & Associates, Flagstaff, Arizona, USA) was positioned across the level of injury. The nail was removed by gentle traction by trauma surgery with the simultaneous deployment of the Gore Viabahn covered stent into the left internal carotid artery spanning the injured segment. Follow-up angiography demonstrated excellent flow through the stent but with active extravasation secondary to a proximal endoleak related to incomplete apposition of the proximal stent to the vessel wall (Figure ).
At this time, the anaesthesia team noted blood pooling in the oropharynx. We introduced a 5 x 30 mm Aviator Plus balloon (Cordis, Milpitas, California, USA) and performed angioplasty of the proximal end of the stent. Follow-up angiography demonstrated no active extravasation and excellent flow through the stent (Figure ).
Cerebral angiography demonstrated no abnormal vessel dropout in the left anterior circulation. There was no further bleeding in the oropharynx or through the tract. The patient was transferred to intensive care unit (ICU) for further care.
On a postoperative day one, the patient was extubated without incident. No neurological deficits were noted. The patient was continued on 325 mg of Aspirin and 75 mg of clopidogrel daily. The patient was cleared for an oral diet. Follow-up CT angiogram demonstrated patency of the left internal carotid stent and no pseudoaneurysm formation (Figure ). The patient did receive tetanus boosters and antibiotics as part of the initial management to prevent secondary infection. The patient was discharged to home on hospital day five. We planned to follow up the patient with CT angiogram at six weeks after the procedure. Unfortunately, we were not able to obtain the imaging as the patient was out of state and was uninsured. Per phone conversation the patient was doing very well and did not report any symptoms consistent with stroke or transient ischemic attack (TIA) at two months after the procedure. The patient stopped his clopidogrel one week after the procedure due to financial reasons but continues on the aspirin regimen. |
pmc-6516627-1 | A 47-year-old Caucasian male with a past medical history of hypertension, hyperlipidemia, and everyday tobacco use presented with left lower extremity pain for over 10 years. The pain, described as a throbbing and cramping sensation without immediate relief after rest, had worsened significantly over the past two years, leading him to seek further medical attention. Prior to the onset of lower extremity pain, the patient was healthy and led an active lifestyle. On exam, his feet were warm and pink with normal sensation and 2+ distal pulses.
Arterial Doppler of the left lower extremity noted severe stenosis of the left superficial femoral artery and popliteal artery. He was started on cilostazol 100 milligrams two times per day, but his symptoms did not improve. Arteriography revealed normal iliac, common femoral, and superficial femoral arteries without atherosclerosis. There was occlusion of the left popliteal artery and extensive collateral flow to the leg. This raised suspicion for PAES and provocative maneuvers were performed during the study. Dorsiflexion and plantar extension of the ankle caused the cessation of the collateral and distal popliteal artery flow. When the ankle was returned to neutral position, the occlusion ceased and flow was restored. These findings confirmed the diagnosis of PAES with resulting popliteal artery occlusion (Figure , Video ). A popliteal artery bypass with a reverse great saphenous vein was performed, and the patient’s symptoms resolved. |
pmc-6516628-1 | A 37-year-old Yemeni male patient was brought to the hospital by his coworker after the patient had been disoriented for several days. A report from another medical center indicated the patient was diagnosed with tuberculosis (TB). According to this report, he had presented to the other facility one month previous with fever, productive cough, and disorientation. He was advised to start anti-TB therapy (ATT). However, the lack of collateral history made it impossible to confirm whether he had been receiving ATT. At presentation to our center, he was drowsy but arousable to strong verbal commands. He uttered a few incomprehensible sounds, and he obeyed spoken commands only occasionally. He appeared to have mild weakness in his left arm. His cranial nerve examination showed no abnormalities. The results of his systemic examination were unremarkable: we noted no skin rash, indications of intravenous drug use, evidence of peripheral stigmata of infective endocarditis, or lymphadenopathy. On subsequent days, he sustained several attacks of generalized tonic-clonic seizures. Given that he was febrile with evidence of meningismus, he underwent lumbar tap.
Investigations
His cerebrospinal fluid (CSF) was light yellowish with 373 cells (73% monomorphic) with glucose at 2.4 g/dL, protein at 620 g/dL. The CSF was positive for JC virus DNA with a viral load of 2800 copies/mL. His serology for human immunodeficiency virus (HIV) was positive with a CD4 count of 135 /µL and an HIV viral load of 179,795 copies/mL. Test results for hepatitis B virus and sputum acid-fast bacilli were negative. CSF polymerase chain reaction results for TB were negative, so was the culture for TB in the CSF sample. However, the report from the peripheral center had shown positive TB culture in CSF. Computed tomography (CT) of the patient’s brain revealed left basal ganglia hypodensity and left periventricular white matter hypodensity. Magnetic resonance imaging (MRI) showed bilaterally scattered patchy confluent subcortical and periventricular foci confirmed on T2-weighted images (T2WI) and fluid-attenuated inversion recovery (FLAIR) images (Figure ). We noted diffusion-weighted imaging (DWI) restriction at the left frontal lobe, bilateral basal ganglia as well as in the brainstem and cerebellar vermis. The largest area of DWI restriction was seen over the right basal ganglia lesion which also showed faint heterogeneous enhancement in the post-contrast images with mild mass effect on the adjacent right lateral ventricle.
Treatment
Based on clinical, imaging, and virological finding, we decided to treat the patient with combined antiretroviral therapy (cART) along with the ATT. Given the reported cases of successful treatment with an anti-5HT2A antagonist, we treated the patient with mirtazapine along with standard cART and ATT.
Outcome
The patient remained clinically stable with no apparent worsening of his neurological deficit. He became more alert and could reply to one or two questions coherently. He sustained no further seizures. He developed rigidity in all limbs and bradyphrenia. He also became hypophonic and apathetic. A second MRI after six weeks of therapy revealed remarkable improvement (Figure ). After six months of treatment, the patient is more alert, obeys commands, and can communicate with two to three sentences at a time. His weakness has resolved, but he has residual rigidity over his limbs. |
pmc-6516630-1 | A 30-year-old G1P0010 presented to the emergency department with a six-day history of abnormal uterine bleeding. Three months prior, she underwent a therapeutic abortion followed by dilatation and curettage (D&C) for retained products of conception. Since then, she was noted to have new-onset menorrhagia, which on the day of presentation became persistent and was associated with severe pain, weakness, and dizziness. She was found to be hypotensive and tachycardic on presentation with marked tenderness in the suprapubic area with an otherwise normal physical exam. Laboratory studies revealed hemoglobin of 9.2 g/dL and hematocrit of 27.5%, and negative beta-human chorionic gonadotropin (beta-hCG).
Transvaginal ultrasound revealed a 3.9 cm x 2.7 cm x. 1.8 cm sized anechogenic cystic space in the posterior wall of the fundus as seen in Figure , and with Doppler application, demonstrated marked vascularity (Figure ). For further evaluation, pelvic magnetic resonance imaging (MRI) was performed, which revealed a contrast-opacified structure within the wall of the myometrium. Focal serpiginous flow voids were also noted within the posterosuperior myometrium with extension to the myometrial canal (Figure ).
Taken together, a presumptive diagnosis of the uterine AVM was made and the patient was referred to interventional radiology. The uterine angiogram confirmed the presence of an AVM within the posterosuperior wall (Figure ). There were multiple feeding arteries mainly from the left with smaller arteries from the right. Embolization of both uterine arteries was performed with Gelfoam (Pfizer, New York, USA) pledgets to near stasis. The post-embolization arteriogram showed complete embolization of the AVM with slow flow of contrast in both uterine arteries. No immediate complications were encountered. The patient's vaginal bleeding resolved and she was discharged three days later. No recurrence of abnormal uterine bleeding was reported at either the three-week or six-month follow-up visit. |
pmc-6516631-1 | A 71-year-old Caucasian gentleman with a past medical history of chronic obstructive pulmonary disease (COPD) was admitted with shortness of air and productive cough. A CT angiogram of the chest was obtained due to concerns for pulmonary embolism and showed spiculated nodules in bilateral lower lobes; right nodule measured 1.1 cm x 0.9 cm while the left measured 1.5 cm x 1.2 cm. Also noted was right hilar lymphadenopathy with the largest lymph node measuring 2.9 cm x 2.1 cm; no mediastinal or axillary lymphadenopathy was noted. Emphysematous changes of the lungs were also seen. A biopsy of the left lung nodule and a right lymph node fine needle aspiration (FNA) were performed. Pathology results revealed the left lung nodule to be poorly to moderately differentiated nonkeratinizing squamous cell carcinoma. Cytology studies on the right lymph node sample showed highly atypical large lymphocytes with 95% monoclonal B-cells on flow cytometry, concerning for large B-cell lymphoma. After stabilization from respiratory standpoint, the patient was discharged home on supplemental oxygen therapy via nasal cannula at three liters/minute (L/min). His case was discussed in a multi-disciplinary tumor board. Cardio-thoracic surgery team felt he was not a candidate for lobectomy and would need radiation therapy; a referral to radiation oncology was made. A lymph node excision biopsy was planned to establish a definite diagnosis of lymphoma.
Approximately eight weeks following his initial admission, the patient presented with worsening dyspnea with productive cough. He was hemodynamically stable with no increase in supplemental oxygen requirements; however, he was noted to have facial swelling, jugular venous distention, diffuse wheezing with bilateral basal crackles and bilateral pitting pedal edema. Treatment was started for acute COPD exacerbation. Intravenous (IV) furosemide was given as well as given peripheral edema along with prednisone 50 mg daily. A CT chest with contrast was performed; this showed a large, centrally necrotic 18.1 cm x 8.7 cm x 9.7 cm bilateral lobulated infiltrating mediastinal mass involving the entire mediastinum and the right hilum, causing mass-effect upon the heart, superior and inferior vena cava and pulmonary arteries and deforming the airway including trachea and right main bronchus. Significant narrowing of the SVC, the right main and right upper lobe pulmonary arteries was seen as well as of the left brachiocephalic vein. Multifocal consolidations were also noted. Bilateral lung nodules were re-visualized but were stable in size in comparison to prior CT scan. Small pericardial effusion was seen (Figure ).
Blood and sputum cultures were obtained; broad spectrum antibiotics including vancomycin, cefepime, and levofloxacin were initiated for pneumonia in setting of recent hospitalization. The mediastinal mass was presumed to be rapidly enlarging lymphoma. Bone marrow and left supra-clavicular lymph node biopsy was obtained by interventional radiology (IR). Over the next 24 hours, he was observed to have increasing tachypnea; oxygen requirement increased from 3 L/min by nasal cannula (NC) to 10 L/min via a non-rebreather (NRB) mask. On examination, the patient was noted to have audible stridor. He acutely became hypotensive; systolic blood pressure (SBP) dropped from 110 mmHg and above up until this point to below 90 mmHg. The deterioration in his hemodynamic and oxygenation status is depicted in Figure .
He was transferred to the intensive care unit (ICU) and was subsequently intubated. Inotropic support was initiated. An arterial line waveform was suggestive of pulsus paradoxus. Electrocardiogram (EKG) showed sinus tachycardia at a rate of 110 beats per minute (bpm) with low voltage QRS (Figure ).
A trans-thoracic echocardiogram (TTE) was immediately performed which showed moderate-sized pericardial effusion with signs concerning for impending cardiac tamponade including right ventricle diastolic collapse. The patient was urgently taken for diagnostic and therapeutic pericardiocentesis; 160 milliliters (mL) of pericardial fluid was drained followed by placement of a pigtail catheter in the pericardial sac. Following the procedure, the patient became increasingly hypotensive and inotropic support was intensified with addition of epinephrine followed by vasopressin to an already maximum rate of norepinephrine. The IV fluids in periodic boluses were given with temporary improvement in blood pressure each time. A repeat TTE showed only mild pericardial effusion. Over the next few hours, the patient developed worsening swelling of his face, upper chest, and upper extremities. He was also noted to have worsening abdominal distention. Eventually, the patient's urine output declined. Pericardial fluid analysis showed bloody appearance with 71% mononuclear cells; malignant lymphoma cells were noted on cytology. His recent lymph node biopsy results were reviewed and a preliminary read was consistent with aggressive DLBCL. Dose adjusted etoposide, prednisone, vincristine, cyclophosphamide, and doxorubicin (DA-EPOCH) were started promptly by the oncology team who felt that the lymphoma was chemo-sensitive and a rapid response to treatment could be anticipated. Prior to chemotherapy initiation, a renal panel was obtained, revealing metabolic acidosis (bicarbonate levels 14.2 mmol/L, pH 7.022 on arterial blood gas), hyperkalemia (potassium 6.0 mEq/L), hyperphosphatemia (phosphorus 6.3 mg/dL), and hypocalcemia (calcium 6.2 mg/dL). Nephrology was consulted for initiation of continuous renal replacement therapy (CRRT) in setting of oliguria and concerns for tumor lysis syndrome (TLS). His family requested for comfort care measures to be pursued at this point; the patient was extubated palliatively and expired within a few minutes. Later, the patient’s respiratory cultures grew Acinetobacter baumannii which was only intermediately sensitive to cefepime, meropenem, and levofloxacin (Table ). |
pmc-6517238-1 | A 14-year-old girl, presented to our clinic with left knee pain, especially on full extension of the knee, for duration of 6 months. There was no trauma involved. Examination showed lateral joint line tenderness without any ligamentous laxity. Plain radiographs of the knee were normal. Further imaging with magnetic resonance imaging (MRI) scan revealed discoid lateral meniscus with a horizontal tear (
). Diagnostic scope was done and we found an incomplete discoid lateral meniscus with complex vertical tear involving anterior horn with horizontal extension into the midbody (
). Arthroscopic all-inside repair was done without any implant. Postoperatively, the patient's knee was protected with a brace. Postsurgery, 6 weeks, the brace was discontinued and the patient started full weight bearing without pain. At 6 months postsurgery, the patient was pain free and without any mechanical symptoms.
The position was supine, with the leg hung freely at the end of the table. A tourniquet was used to secure hemostasis. A standard anterolateral portal was used for diagnostic arthroscopy. The finding was an incomplete discoid lateral meniscus with peripheral vertical tear of the anterior horn and partial horizontal tear involving the mid body but not breaching the inner peripheries (
). A standard anteromedial portal was made and the tear was further assessed using a probe. The tear site was prepared for repair. An additional far medial portal was made to allow arthroscopic instrumentation. A suture passer loaded with synthetic monofilament absorbable suture, introduced through far medial portal, while viewing through anteromedial portal (
). The torn central fragment and the peripheral rim of the meniscus were penetrated and the tip of suture was pulled through using an arthroscopic grasper through anterolateral portal. The suture passer was reversed out of the far medial portal, leaving the suture inside. Then, a suture retriever was introduced through anterolateral portal and used to retrieve the suture tip bringing both suture-ends out through one portal. Both the suture ends were tied using a sliding knot technique. This whole process was repeated for a second suture repair (
). The stability of the repair was assessed using a probe. Initially, we planned to saucerize the discoid meniscus together with meniscus repair. However, intraoperatively we decided not to saucerize the meniscus in view of the tear configuration. Due to the complex nature of the tear pattern (peripheral vertical tear with extensive horizontal tear through the meniscus body), saucerization would have made the articular surface of the meniscus a loose fragment or created a flap tear, which is more difficult to repair. |
pmc-6517458-1 | A 71-year-old male with a history of alcoholism and radiation treatment (total dose of 57.6 Gy) for early-stage vocal cord cancer 16 months earlier was referred to us with radiographic detection of a pulmonary nodule in the right lower lobe. Endobronchial biopsy of the pulmonary nodule revealed the diagnosis of squamous cell carcinoma. Positron emission tomography/computed tomography (CT) revealed accumulation in the pulmonary nodule, but no other lesion was suggestive of metastasis. The tumor was diagnosed as a primary lung cancer, clinical stage IA1 (T1aN0M0), or metastatic pulmonary tumor, and video-assisted thoracoscopic right lower lobectomy with lymph node dissection was performed. Level 8 and 9 lymph nodes were not enlarged; therefore, lymph node dissection of these nodal station was not performed (Fig. a), while the level 7 lymph nodes were dissected. There was no direct injury of the esophagus, and no apparent esophageal damage was visualized on magnified video endoscopic images (Fig. b). The operation was uneventful. The patient was able to resume oral intake from the day after the surgery.
The patient tended to need to make a strong effort to cough, with swallowing and expectoration having become difficult, presumably due to the radiation therapy given to the larynx. Three days after the operation, the patient developed severe sudden pain in the right shoulder with high fever (39.6 °C). Findings of the chest X-ray obtained with a portable apparatus showed a few infiltrative shadows in the right lung field, and blood examination revealed no findings that were not compatible with the postoperative status of the patient. The patient was started on intravenous antibiotic administration. Absence of air leakage through the chest tube was confirmed, and the chest tube was removed 4 days after the operation. However, a plain chest X-ray revealed increased infiltrative opacities in the right lung field, and the patient developed a right pneumothorax 5 days after the operation. A chest tube was re-inserted into the right pleural cavity. Sputum mixed with a small amount of blood and cloudiness of the discharge from the right chest tube was confirmed 7 days after the operation. Chest CT showed a marked increase in the size of the right pleural effusion, with air bubbles visualized within the opacity. Although suture failure at the bronchial stump could not be confirmed by bronchoscopy, empyema due to BPF was suspected at first, and open-window thoracostomy (OWT) of the right chest was performed.
Two days after the OWT, contamination of the dressing by food particles was confirmed. An esophagogram revealed communication between the lower portion of the esophagus and the right thoracic cavity (Fig. a). An upper gastrointestinal endoscopic examination also showed a tiny orifice in the lower portion of the esophagus (Fig. b). A nasal W-ED tube™ (Nippon Covidiene Inc., Tokyo, Japan) for simultaneous gastric compression and jejunal alimentation was inserted under endoscopic guidance into the jejunum, and oral intake was totally prohibited.
Dressing changes twice daily were continued, until culture of the thoracic drainage fluid no longer grew pathogens. Omentopexy was performed 20 days after the OWT. A small longitudinal tear, about 1.5 cm in length, was identified in the lower portion of the esophagus (Fig. a), below the inferior pulmonary vein stump. No pulmonary fistula formation with esophagus or any BPF was identified. Although the edge of the tear was comparatively clear, the omental pedicle flap (OPF) was sutured around the tear without direct suture, because several days had elapsed after the onset of the esophageal rupture (Fig. b). Two thoracic drainage tubes were inserted into the thoracic cavity and the wound was finally closed. The postoperative course was uneventful. An esophagogram obtained 7 weeks after the omentopexy showed no leakage of contrast medium through the esophageal wall (Fig. a). The patient was discharged 13 weeks after the operation. A chest CT performed 5 months after the operation revealed complete healing of the EPF and resolution of the empyema of the right thoracic cavity (Fig. b). |
pmc-6517474-1 | A 75-year-old woman was admitted to our palliative ward with abdominal pain, nausea, and vomiting. The patient had been diagnosed with metastatic pancreas carcinoma with one singular liver metastasis 18 months before. She had received first- and second-line chemotherapy regimens; the latter had been stopped due to severe side effects. Two months prior to admission, when MRI scans revealed progressive disease, and together with her medical oncologist, the patient decided against continuing chemotherapy. Instead, symptom oriented, palliative care was chosen without any further antineoplastic therapy.
The patient had been suffering from RLS for 12 years already, with moderate to strong symptoms [Numerical Rating Scale (NRS): 6-10/10] mostly in the evening and at night. The family history regarding RLS was not investigated. She reported symptom alleviation by long walks (up to several hours long), and rigorous tennis playing, both of which she could no longer accomplish because of the progressive cancer related fatigue. Twelve years ago, her neurologist started treating RLS with levodpa, but after initial improvements in symptom control, symptoms began worsening again due to augmentation. Five years later, the patient was started on a transdermal application of the dopamine-agonist rotigotine (4 mg/d), but this treatment could not reduce RLS-symptoms satisfactorily. The patient reported that a trial of pregabalin was discontinued because of side effects (dizziness) and oxycodone was stopped because of nausea and vomiting. Thereafter transdermal rotigotine (4 mg/d) was continued with little effect until admission to our palliative care unit.
To assess RLS-symptom burden and pain we used the 11-NRS, an established tool to assess pain and commonly used in the palliative care setting, where 0 = no pain and 10 = worst possible pain (). We used the NRS to semi-quantify RLS-symptom intensity, because it is well-known by staff while other assessment tools specifically designed for RLS are not established. When using the NRS for the assessment of RLS-symptoms, we asked the patient: how severe are your RLS-symptoms right now (0 = no RLS-symptoms, 10 = worst possible RLS-symptoms)?
Upon initial admission, she reported abdominal cramps (NRS 8/10). Her temperature and blood pressure were normal with a heart rate of 100 bpm. The abdomen was distended, but soft with normal bowel sounds. The patient reported ubiquitous abdominal tenderness. The rest of the physical examination was normal. Initial laboratory testing included elevated gamma-glutamyl transferase at 194 U/l (reference range, <40 U/L), lactate dehydrogenase at 364 U/L (reference range, 135–214 U/l) and C-reactive protein at 42 mg/l (reference range, <5 mg/l). Bilirubin and lipase levels were normal. The peripheral-blood count was normal. An abdominal ultrasound dismissed possible bowel obstruction, hepatic cholestasis, and gall bladder abnormalities, but revealed a significant amount of ascites, which is why percutaneous ascites drainage was performed (3.5l). Cell counts in the ascites fluid revealed elevated neutrophils/μl indicating spontaneous bacterial peritonitis. Calculated antibiotic treatment was started with tazobactam/piperacillin.
The patient also received intravenous fluids, analgesics (oral metamizole) and antiemetics (dimenhydrinate, ondansetrone). At day 5 after admission the abdominal pain exacerbated. Symptomatic analgesia with intravenous morphine (20 mg/d) was initiated. Pain management was excellent after 1 day with a NRS of 0-3/10. Unintendedly, the patient also reported almost complete symptom relief regarding her RLS (), which had not occurred for her in years. After nausea and vomiting had resided, analgesics, including morphine were given orally. Still, pain management and the symptom control of RLS-symptoms remained steady. According to the patient's wish, she was discharged 13 days after admission. Three days later, she was re-admitted with increasing abdominal pain. Without our knowledge, her general practitioner had rotated morphine to transdermal fentanyl (25 μg/h). While pain control was insufficient, RLS-symptoms remained adequately controlled with this opioid therapy. After re-admission we discontinued transdermal fentanyl and re-initiated intravenous morphine therapy, which once again achieved excellent pain relief. Paracentesis revealed an increasing neutrophil count in the ascites. Considering her incurable, advancing and metastatic disease and good symptom control under analgesia, the patient declined antibiotic treatment and died a little more than 1 week later. |
pmc-6517487-1 | A 21-month old male presented with hypotonia and global developmental delay (; ). At 21 months, he sat supported, was non-verbal but understood “no” and could wave as a gesture to communicate goodbye. He had intermittent upward eye deviation, diagnosed as oculomotor apraxia, head drops, hand twitching, and continued episodes of staring. Continuous EEG monitoring was abnormal with a slow background and multi-focal spike and wave but without electrographic changes with the abnormal movements. He had delayed myelination on brain MRI at 9 months old with mild increased T2 signal in the bilateral thalamus (). CSF studies for neurotransmitters (excluding GABA) and amino acids were normal. Whole exome sequencing revealed compound heterozygous variants of uncertain significance (VUS) inherited in trans in ABAT (c.454C > T, p.P152S and c.1393G > C, p.G465R). Diagnosis was confirmed with CSF GABA elevated at 247 nmol/L (normal range 17–67 nmol/L). |
pmc-6517487-2 | Patient 2 is a 6-year old male (at the time of testing) previously reported with GABA-transaminase (). He presented initially with hypotonia and vision impairment. He had severe progressive psychomotor retardation with inability to even hold his head, non-verbal, medically refractory intractable seizures, and cortical visual impairment. His initial EEG was normal early in infancy but progressed with generalized slowing and multifocal spike and wave activity. MRI at age 17 months reported severe global atrophy involving the right cerebral hemisphere more than left and signal abnormalities involving bilateral internal capsules and dentate nuclei and decreased amount of white matter, as well as delayed myelination (). He had similarly affected sisters who died at age 9 years and 1 year old. Whole exome sequencing revealed a homozygous VUS in ABAT, c.631C > T (p.L211F), later shown to result in enzyme deficiency (). |
pmc-6517487-3 | Patient 3 is a 4-year old male, born by C-section due to fetal macrosomia. Evaluation at 4 years of age revealed motor delays (walked independently at 3 years of age) and speech delays (20–30 words), mild hypotonia, significantly ataxic gate (frequent falls), autistic features (behavioral outbursts in unfamiliar settings, hypersensitive to noise and aversion to anything touching his head) and strabismus. Brain MRI revealed abnormal increased signal in the T2 imaging in thalami, brainstem, globus pallidus, and cerebellar dentate nuclei bilaterally, as well as in deep and subcortical white matter (). No seizures have been reported to this date. Whole exome sequencing identified two variants inherited in trans in ABAT: a c.168+1G > A likely pathogenic variant and heterozygous c.638T > G (p.F213C) VUS. No other significant findings were present in this patient at the time of evaluation. |
pmc-6517487-4 | Patient 4 is a 7-year-old female who initially presented at age 5 months with hypotonia, failure to thrive, and global developmental delay. Generalized chorea, characterized by writhing movements of all limbs and tongue thrusting, emerged between 6 and 12 months. She also developed multifocal jerky movements suggestive of myoclonus. Her movement disorder is partially controlled with clonazepam and levetiracetam. She has profound developmental delay, with absent head control and a general paucity of purposeful voluntary movements. There is no history of clinical seizures. EEG at age 3 years demonstrated generalized slowing, and a repeat study at 7 years showed multifocal sharp waves. Brain MRI at age 6 years demonstrated frontally predominant cerebral atrophy, bilateral frontal periventricular nodular heterotopia, thick corpus callosum, and abnormal areas of T2 hyperintensity in bilateral thalami and midbrain (). The patient is the product of a close consanguineous union. Whole exome sequencing detected a homozygous VUS in ABAT, c.1394G > A, p.G465D. In addition, the patient is homozygous for a VUS in LRRC7, c.2938C > T, p.R980X. LRRC7 has not been associated with a human disease. Diagnosis of GABA-transaminase deficiency was confirmed with significantly elevated levels of free and total GABA in CSF: 272 (nM) and 32.2 (μM), respectively (reference ranges: free GABA, 32–170 nM and total GABA, 3.3–12.2 μM) (Baylor Institute of Metabolic Disease, Dallas). |
pmc-6517493-1 | Mrs. A was a 66-years-old right-handed woman who suffered a stroke the day of her professional retirement, causing her two simultaneous hemorrhagic lesions that affected frontal and parietal areas in the left hemisphere, partially sparing the core perisylvian language areas (see ). Mrs. A was a monolingual native Spanish speaker with normal language development during childhood. She was a highly educated woman (24 years of formal education), previously working as a full professor of mathematics at a Spanish University. Mrs. A was referred to our unit for aphasia evaluation 27 months post-onset. At that moment, she had a mild right hemiparesis, right visual field defect and a severe aphasia. Mrs. A had a severe non-fluent aphasia characterized by markedly reduced spontaneous speech and almost nil auditory comprehension in the face of preserved language repetition (MTCA) (). Note that this type of aphasia is equivalent to global aphasia, except for the preservation of repetition capacity. Automatic echolalia, verbal perseverations, and ready-made expressions were very frequent in Mrs. A. Further, she could sing overlearned songs without prompting using excellent lyrics and melody.
Several episodes of LA were detected in Mrs. A throughout the language evaluations. During language testing sessions she would frequently get frustrated and when she was not able to perform a linguistic task, she blocked out and reiteratively said “I don’t know, I don’t know, I don’t know…”. After that, the evaluation had to be stopped, jumping to the next task since she refused to continue with that task. In other occasions, when she was blocked out with an item that she did not understand or was not able to name, she would go back to the items, even after several items had passed, and tried to get feedback (on the name or meaning) from the therapist. This was usually accompanied by face expressions and body gestures indicating worry and tension. We observed that during testing her verbal behavior was replete with echolalic emissions, which markedly increased when she was anxious and upset. However, Mrs. A’s husband reported that echolalia was not excessively pronounced in other contexts (e.g., home, interactions with family members). The presence of severe LA and worsening of language performance when Mrs. A was assessed in a clinical setting by a therapist jeopardized the interpretation of cognitive evaluations, leading to biased conclusions about the severity of the language alteration and the effectiveness of the treatments. However, when language evaluation was performed by Mrs. A’s husband her performance improved significantly. |
pmc-6517511-1 | An 82-year-old woman being treated for pyelonephritis was referred to our hospital because of a nodule in the left anterior segment of the lung on chest CT. The nodule was part solid, 1.9 × 1.1 cm in size and reveal a pleural tag (). The maximum standardized uptake value of the tumor is 5.7 by 18F-fluorodeoxyglucose-positron emission tomography. The nodule was not confirmed by any histopathological examination; however, it was strongly suspected to be lung adenocarcinoma. Brain magnetic resonance imaging and abdominal CT revealed no obvious metastases. The clinical stage was T1bN0M0 stage IA2 as categorized by the UICC TNM Classification (8th edition). Spirometry demonstrated an FEV1.0 of 1.47 L and FEV1.0% of 53.4%. Moreover, the cardiac function was maintained to be normal.
We recommended the patient to undergo a lung resection for the diagnosis and treatment of the nodule, and she agreed. We believed that this lesion could be completely resected by anterior and lingular segmentectomy based on preoperative imaging diagnosis. However, if the nodule is not palpated, then a sufficient tumor margin may not be secured; thus, we decided to perform a CT-guided nodule marking prior to the surgery. |
pmc-6517850-1 | A 12-year-old girl presented with a 2-year history of swelling of the medial lower lid and persistent discharge in the right eye. She reported that during this time, she had seen different ophthalmologists and her symptoms had improved slightly with medical treatment, but never completely resolved. Review of her hospital records showed that she had been prescribed various antibiotic and steroid eye drops and ointments for diagnoses of conjunctivitis, chalazion, and lacrimal duct stenosis. External examination of the right eye revealed thick purulent secretion and swelling in the punctum area of the medial lower lid (). The lower punctum was enlarged and compression resulted in purulent secretion from the punctum. The lacrimal duct was patent upon irrigation. However, the presence of dacryoliths was felt as the cannula tip was inserted into the lacrimal duct.
Based on the examination findings, the patient was diagnosed with canaliculitis. Due to her history of poor response to long-term medical treatment, we decided to remove the dacryoliths surgically. The patient was admitted for surgery under general anesthesia. We first attempted to spare the canaliculus and remove the dacryoliths by expanding the punctum with a dilator. When this failed to provide a large enough opening, a one-snip punctoplasty was performed. A chalazion curette was used to completely remove the dacryoliths ( and ). The lacrimal system was washed with 5% povidone-iodine solution (Batticon). Postoperatively, the patient was given topical 100,000 U/mL crystallized penicillin 8 times a day for 10 days. The removed dacryoliths were sent for histopathological and microbiological examination. Histopathology revealed sulfur granules associated with Actinomyces (). Hyphal structures consistent with Actinomyces were observed in Gram staining, but culture was negative. At the patient’s last follow-up 12 months later, her symptoms had completely resolved with no recurrence. |
pmc-6517852-1 | A 27-year-old woman presented to our clinic in 2008 with progressive visual impairment in both eyes. On ophthalmologic examination, her best corrected visual acuity (BCVA) on Snellen chart was 0.3 (-4.50) in the right eye and 0.2 (-4.50) in the left eye. Slit-lamp examination showed clear cornea, calm anterior chamber, and transparent lens in both eyes. Deposits were not observed in the corneal limbus of either eye. The optic discs appeared normal on fundus examination. Extensive shiny white-yellow deposits were observed in the posterior pole and mid-peripheral retina ( and ). Based on these findings, a clinical diagnosis of BCD was made and the patient was scheduled for follow-up.
Upon retrospective analysis of her records, we noticed that patient did not undergo OCT in 2008. OCT (Spectralis; Heidelberg Engineering, Heidelberg, Germany) performed in 2014 revealed central macular thickness was 194 µm in the right eye and 198 µm in the left eye. Hyperreflective intraretinal spots were observed in the sensorial retina and hyperreflective plaque-like deposits were identified at the RPE-Bruch’s membrane junction. Intraretinal cystic spaces were observed in some of the sections. Outer retinal tubulation was noted in the outer retinal layers. Choriocapillaris atrophy and subsequent enhanced visibility of the large choroidal vessels was noted in enhanced depth imaging mode, which provides better visualization of the choroid. Complete obliteration of the choroidal vasculature was observed in some places. Choroidal hyperreflective foci were noted around the choroidal vessels ( and ). Swept-source OCT (SS-OCT) performed in 2018 showed a relative reduction in the intraretinal hyperreflective spots and hyperreflective plaque-like deposits at the RPE–Bruch’s membrane detected in 2014 ( and ).
On examination in 2018, BCVA was 0.1 (-4.50) in both eyes. There were still no signs of corneal pathology on slit-lamp examination. Fundus examination revealed retinal crystalline deposits and extensive areas of retinal and choroidal atrophy in both eyes ( and ). In SS-OCTA (Topcon DRI OCT Triton, Topcon, Japan), the choroidal vessels were visible in the area of outer retinal layer projection due to increased permeability resulting from diffuse RPE atrophy; in the area corresponding to the choriocapillaris projection, no flow associated with the choriocapillaris was observed and vessels belonging to the deeper choroidal layers were apparent in this area (, , , and [right eye]; , , , and [left eye]). |
pmc-6517856-1 | A 58-year-old woman presented with complaints of decreased visual acuity and metamorphopsia in her left eye. Her history was unremarkable and full ophthalmological examination was normal. Color fundus and red-free fundus images (Topcon SD-OCT; Topcon Medical Systems, Paramus, New Jersey, USA) were normal (). Best corrected visual acuity (BCVA) of her left eye was 8/10 and SD-OCT (Topcon SD-OCT; Topcon Medical Systems, Paramus, NJ, USA) revealed incomplete PVD and VMT. There was puckering and disorganization of the inner retinal layers due to anteroposterior traction, irregular foveal contour, and a defect approximately 140 microns wide in the external limiting membrane (ELM) and photoreceptor inner segment–outer segment (IS/OS) layers ().
After 5 months of follow-up, the VMT spontaneously regressed, after which the patient’s metamorphopsia resolved suddenly, BCVA in that eye increased to 9/10, and SD-OCT revealed complete normalization of the foveal contour as well as regression of the irregularities in the inner retinal folds. In addition, an operculum was observed over the macula attached to the residual posterior hyaloid membrane, and a defect 90 microns in diameter persisted in the ELM and IS/OS layers (). At 46-month follow-up, the patient was asymptomatic and the defect in the outer retinal layers was found to persist at a size of 68 microns. The operculum on the detached posterior hyaloid membrane over the macula was also visualized using three-dimensional (3D) SD-OCT (). |
pmc-6517856-2 | A 65-year-old woman presented due to sudden-onset metamorphopsia in her left eye. BCVA was 4/10 in the affected eye and color fundus and red-free fundus images (Topcon SD-OCT; Topcon Medical Systems, Paramus, New Jersey, USA) were normal (). Examination of the left eye with SD-OCT (Topcon SD-OCT; Topcon Medical Systems, Paramus, NJ, USA) revealed grade 3 PVD and an operculum over the macula, as well as a 156-micron outer retinal defect in the ELM and IS/OS (). The operculum on the residual posterior hyaloid membrane over the macula was also observed in 3D SD-OCT ().
At 42-month follow-up, BCVA in the affected eye was 6/10 and a 90-micron defect persisted in the ELM and IS/OS (). |
pmc-6517856-3 | A 59-year-old man presented due to central scotoma that had recently developed in his right eye. BCVA was 8/10 and infrared fundus images (Heidelberg Spectralis HRA+OCT; Heidelberg Engineering, Heidelberg, Germany) showed a dark spot in the fovea (). SD-OCT (Heidelberg Spectralis HRA+OCT; Heidelberg Engineering, Heidelberg, Germany) revealed grade 3 PVD with an operculum attached to the posterior hyaloid membrane remnants overlying the macula, and a 122-micron defect in the ELM and IS/OS layers ().
After a 10-month follow-up period, the patient had BCVA of 9/10, persistent central scotoma, and the outer retinal defect was unchanged (). |
pmc-6517857-1 | A 22-year-old woman was referred to Gazi University, Department of Ophthalmology with photophobia and redness in both eyes starting one week earlier. Best corrected visual acuity was 20/20 in both eyes, although she described discomfort with her vision. Slit-lamp examination revealed bilateral conjunctival injection and anterior chamber reaction which was graded as +4 accompanied by fine, non-granulomatous bilateral keratic precipitates (). Dilated fundus examination demonstrated normal retinal findings, with no vascular sheathing or any sign of retinitis (). Optical coherence tomography (OCT), enhanced depth imaging-OCT, and fundus autofluorescence (FAF) were all normal (). In addition to her ophthalmic symptoms, the patient had redness and pain in her right ear. Physical examination of the patient showed cartilaginous inflammation of the right ear (). The patient was referred to the rheumatology department for further systemic evaluation. Hematological examination demonstrated elevated serum erythrocyte sedimentation rate and C-reactive protein level (69 mm/hr ve 126 mg/L, respectively). Complete blood count and other biochemical parameters were within normal ranges. Infective and inflammatory markers were also normal (anti-DNA, ANA, C3 and C4 immunoglobulin, anti-SSA, anti-SSB, anti-SM, anti-SCL, and anti-JO). The patient was treated with topical dexamethasone 0.1 mg/5 mL ophthalmic solution hourly, cyclopentolate 1 %3 times a day, and systemic oral 1 mg/kg/day prednisolone therapy with a plan to taper.
After one month of this combination of topical and oral steroid therapy, her best corrected visual acuity was stable and visual deterioration was resolved. Slit-lamp biomicroscopy revealed a dramatic regression in the anterior chamber reaction, with only trace anterior chamber cells/flare and few keratic precipitates (). Treatment continued with slow tapering. |
pmc-6518154-1 | A 69-year-old married woman with a previous clinical history of both primary breast and endometrial cancers was referred to the Cancer Rehabilitation Center (Ce.Ri.On) in Florence for cancer-related follow-up management. The survivor was included in the Ce.Ri.On waiting list for possible rehabilitation programs, and then randomly recruited to initiate APA intervention. Tracing the clinical history, in May 2008 she was diagnosed with breast cancer (i.e., ductal carcinoma in situ) and underwent bilateral mammoplasty after right lumpectomy with radiation therapy. Moreover, in August 2015 after a diagnosis of invasive endometrial adenocarcinoma, the patient underwent total extrafascial hysterectomy by laparoscopic approach, bilateral ovariosalpingectomy and bilateral pelvic lymphadenectomy removing ten and eight lymph nodes at left and right side, respectively. Subsequently, the patient was treated with combinations of chemotherapy and either internal or external pelvic radiotherapy. Following radiotherapy, LLL developed. About two years after hysterectomy, the patient underwent further surgery for local recurrent disease with removal of the pelvic back wall without adjuvant treatment. In December 2017, on the basis that there was no medical contraindication, the Ce.Ri.On rehabilitation physician recommended the woman’s participation in a well-planned and structured PA pathway (i.e., eight weeks of APA followed by twelve weeks of adapted fitness (AF)) to attenuate and improve cancer- and treatment-related problems with particular attention to LLL. Lymph drainage was also scheduled to treat LLL, but it was not performed because of patient’s personal reason at the call time. Therefore, the woman participated in a structured PA intervention without prior physiotherapy management.
Physical examination detected more pronounced left LLL and fibrosis than at the contralateral lower limb. Physical assessment and the eight-week APA program were carried out at the Ce.Ri.On center between January 2018 and March 2018. After ending APA, an AF protocol was properly continued outside the Ce.Ri.On from April to June 2018. At baseline, during and after ending the structured PA intervention, the woman underwent a functional test battery to assess the mobility of upper limb joints (active ROM test and muscle length test) and the flexibility of the spine (sit-and-reach test) [,,]. In particular, active ROM was evaluated by goniometry with the subject standing and taking into account the extension (normal reference range 0–45), flexion (normal reference range 0–180), abduction (normal reference range 0–180), and external rotation (normal reference range 0–90). Shoulder mobility was assessed with the muscle length test executed with the subject in a supine position by elevating the arm and measuring the distance (cm) from the lateral epicondyle to the surface (i.e., a smaller distance from the surface corresponding to a better mobility function). The sit-and-reach test is an indirect measure of both hamstring musculotendinous unit length (indirect measure of hip ROM) and of lumbar ROM. To perform the test, the subject sat with extended legs as straight as possible, then flexed their hip joints and vertebral column to reach forward as far as possible and touched their toes in dorsiflexion with the fingertips of both hands simultaneously. A meter rule was placed between the legs with 0 cm located at the heel line to measure the distance from toes to hands. Moreover, the subject filled out the Short Form-12 (SF-12) and the numerical rating scale (NRS) questionnaires to assess the QoL and to quantify shoulder, lower limb and back pain intensity, respectively. Finally, both lower limbs were assessed by multiple circumference measurements at several anatomical points from foot to hip with a thin, flexible tape []. A difference between affected and unaffected lower limb girth of ≥3 cm at any measurement point was considered indicative of moderate/severe LLL. Anthropometric parameters, such as height and weight, were also measured at baseline and after ending the structured PA intervention and used to calculate body mass index (BMI, kg/m2). The study was carried out following the rules of the Declaration of Helsinki of 1975 (), revised in 2013. Following that, no ethics committee was needed for this publication and the Italian Protection of Personal Data Law (D. Lgs. n. 196/2003) was complied. A signed informed consent form was obtained from the participant.
The structured PA pathway, planned by an adapted exercise specialist on the basis of the specific patient’s needs, comprised eight weeks of APA followed by twelve weeks of AF. In particular, a reduction of LLL, an increase in the shoulder mobility, and an overall improvement of pain and QoL were considered specific objectives to achieve through the structured PA pathway. The APA protocol consisted of one-hour sessions performed during two nonconsecutive days per week and was organized into three phases as previously described []. In the first phase (from the 1st to the 5th session), breathing exercises and exercises to mobilize the pelvis and stretching were proposed, including specific exercises to preserve or improve upper limb mobility. In the second phase (from the 6th to the 12th session), the upper limb mobility exercises of the first phase were replicated, increasing the working time. Postural exercises were also proposed according to the active posture reeducation []. The third phase (from the 13th to the 16th session) comprised a circuit training workout including alternated low-intensity and higher workload exercises. Postural exercises were also replicated. Because of the patient’s lower limb impairment, specific exercises to increase ankle and hip active ROM and, therefore, make the subject able to start executing squats and lunges were also included in the second and third phases. All sessions started with a 10-min warm-up of general exercises and ended with a 10-min cool-down of stretching and breathing exercises. After ending the eight-week APA, the woman voluntarily participated in an AF program which is proposed by Ce.Ri.On as enhanced PA to increase physical fitness and then possibly start a sport activity. In particular, the twelve-week AF program was performed in one-hour sessions during two nonconsecutive days per week. PA was divided into three phases and each phase consisted of warm-up, adapted training and cool-down as detailed in . Each exercise was carefully described through simple biomechanical concepts and visually shown by the exercise specialist. The warm-up and the cool-down phases were the same for all fitness program phases. The warm-up consisted of 20 min of general aerobic exercises for major muscle groups while the cool-down included 15 min of stretching and breathing exercises. The central adapted training lasted about 25 min and was organized as follows. In the first phase (from the 1st to 3rd week), the AF program was aimed to provide continuity to the activities previously executed during APA. First, specific walking pattern exercises were proposed. Ankle and hip mobility exercises were to be performed either in a standing position or on the floor. In addition, exercises such as squats and lunges were continued. Beginner core exercises were also proposed. In the second phase (from 4th to 8th week), lower limb resistance and strength training were included (i.e., exercises for hip and leg flexor, extensor, abductor, and adductor muscles). Throughout the four weeks a progressive resistance exercise program was applied, and thus all bodyweight exercises were performed in 3 sets of 5–12 repetitions. Beginner core exercises were replicated. In the last phase (from the 9th to 12th week), coordination and balance exercises were proposed in circuit training to increase the difficulty of each exercise. Moreover, exercises included the use of resistance bands and a small gym ball, in order to improve the fitness performance. Core exercises were implemented. Each exercise was carefully described through basic biomechanical concepts and visually shown by the exercise specialist. The patient engaged well with all the proposed sessions and, therefore, the adherence to the PA pathway was complete (i.e., the patient attended 100% of scheduled PA sessions). |
pmc-6518441-1 | A 57 year old female from Sindhuli, Nepal, with no known medical comorbidities other than smoking, presented to the emergency department with two episodes of hematemesis over 24 hours. She had a 4-month history of dyspepsia, melena and fatigability. On physical exam, she was pale, blood pressure was 90/50 mm Hg with a heart rate of 130 beats per minute. Other examinations, including abdominal exam, were unremarkable. She was resuscitated with intravenous fluids and packed cell transfusion.
Laboratory parameters with normal ranges in parenthesis, are as follow:
Complete blood count before transfusion: white cell count 7.9 (4–10) × 10
9/L; neutrophils 70%; lymphocytes 26%; monocytes 4%; red blood cells 2.8 (4.2–5.4) × 10
12/L; haemoglobin 9 (12–15) g/dL; platelets 295 (150–400) × 10
9/L.
Biochemistry: random blood sugar 124 (65–110) mg/dL; urea 49 (17–45) mg/dL; creatinine 0.9 (0.8–1.3) mg/dL; sodium 140 (135–145) mmol/L and potassium 4 (3.5–5) mmol/L.
Hepatic panel: bilirubin total 1 (0.1–1.2) mg/dL and direct 0.6 (0–0.4) mg/dL; alanine transaminase 35 (5–30) units/L; aspartate transaminase 40 (5–30) units/L; alkaline phosphatase 98 (50–100) IU/L; albumin 3.5 (3.5–5) g/dL
After stabilization, she underwent upper gastrointestinal endoscopy the same day which revealed an ulcer (10 mm × 6 mm) in lesser curvature of the stomach without active bleeding. The provisional diagnosis was gastric ulcer due to
H. pylori infection. The main differential diagnosis was gastric carcinoma, hence biopsy was taken from the ulcer.
She was started on triple therapy regimen empirically for
H. pylori eradication containing clarithromycin 500 mg, amoxicillin 1 gm and pantoprazole 40 mg twice daily for 14 days, and discharged. When she returned for follow-up, the histopathology showed fungi with broad ribbon like morphology, fruiting bodies consistent with mucor in the ulcer, as well as in granulation tissue but no necrosis or vascular thrombi (
). It was also positive for
H. pylori in Giemsa staining. She was diagnosed with gastric mucormycosis with
H. pylori coinfection. But the confirmation of mucormycosis via culture or molecular tests could not be done because of unavailability of these tests in our facility. The abdominal imaging was not done because there was no evidence of invasive form of the disease in histopathology.
She was readmitted and started on liposomal amphotericin B, 5 mg/kg on the first day followed by 10 mg/kg for the next 13 days. She completed a total of 2 weeks of therapy without any complication. She had no dyspepsia, melena or hematemesis by then. She was then discharged with posaconazole 300 mg once daily, as an oral step-down therapy. However the blood level of posaconazole to ensure the therapeutic level was not tested due to its unavailability in resource- limited setup in Nepal.
On repeat follow up in 1 month, she was asymptomatic. Repeat endoscopy showed a healing ulcer in lesser curvature of stomach. Repeat biopsy showed absence of fungal hyphae. She took posaconazole for total 2 months and stopped the medicine due to financial constraints. She was doing well when she was last followed up 4 months later. |
pmc-6518648-1 | A 43-year-old Hispanic woman with no past medical or family history of importance, presented to our emergency room (ER) with progressive lumbar pain for the past 4 months, 10/10 in intensity, which irradiated to her left lower limb limiting her functionality. She also referred weakness of her left leg, associated with loss of sensitivity, and had experienced night fevers, chills, and a 23 kg (50 pound) weight loss. A physical examination revealed monoparesis of her left leg, associated with hyperreflexia, and hypoesthesia.
A contrasted pelvis and lumbar magnetic resonance imaging (MRI) showed a solid infiltrative mass in her left sacral and iliac bones, compromising the left sacroiliac joint, the ipsilateral sacral nerve roots, and the pyramidalis and gluteus medius muscles. Other bone lesions compromised the left femoral neck and the right femoral diaphysis (Fig. ).
The hypothesis was that these lesions were metastatic, so further studies were ordered. Breast ultrasonography revealed a mass of 2 cm by 3 cm in her left breast, but a subsequent fine-needle biopsy showed benign histopathology. A computed tomography (CT) scan revealed masses in both her liver and lung (Fig. ). A bronchoalveolar wash was negative for malignancy, and so was a transbronchial biopsy. A decision was made to do a CT-guided percutaneous biopsy of the sacral lesion; the results revealed a metastasized lung adenocarcinoma (Fig. ), negative for ALK mutation but with a complex mutation of the EGFR gene: a 19-Del associated with a T790M (exon 20) mutation. The genetic assay used was cobas® EGFR Mutation Test v2 (Roche®). The target deoxyribonucleic acid (DNA) was amplified and detected on the cobas® 480 system which measures the fluorescence generated by specific polymerase chain reaction (PCR) products, using the amplification and detection reagents provided in the cobas® EGFR mutation test kit (lightmix®).
Stage IV lung adenocarcinoma was diagnosed. In the absence of third-generation EGFR-TKIs (not approved by the local drug and food administration at the time) and taking into account the performance status of our patient, which was Eastern Cooperative Oncology Group (ECOG) 2, platinum-based chemotherapy of gemcitabine (1000 mg/m2 day 1 and 8) with carboplatin (AUC 6) was initiated. Only grade 1 toxicities were observed.
Our patient suffered from a severe headache 25 days after admission, with no response to analgesia, and a CT scan of her brain was done; the CT scan revealed intraparenchymal bleeding on the left cerebellar hemisphere, with fourth ventricle compression and non-communicant acute hydrocephalus. A brain MRI showed a lesion in the cerebellum, thought to be a metastasis (Fig. ). After stabilization of the clinical status, whole brain irradiation with three-dimensional conformational radiotherapy (CRT) was done (3 Gy fractions for a total dose of 30 Gy). Radiation therapy (three-dimensional CRT) was also administered to her left sacral and iliac bones for pain management (4 Gy fractions for a total dose of 20 Gy). She died 4 months after diagnosis. |
pmc-6518656-1 | A 68-year-old Caucasian female presented to our hospital with shortness of breath and unintentional weight loss of 30 pounds three months prior. The patient was an active smoker and her past medical history included well controlled type 2 diabetes mellitus (Hemoglobin A1C 6.0%) and non-obstructive coronary artery disease. Her medication included insulin, aspirin and metoprolol succinate. Thoracic computed tomography showed an interlobular mass in the medial right upper lobe with extension into the right hilum (Fig. ). Tissue biopsy was performed and histopathology was consistent with non-small cell adenocarcinoma with negative EGFR mutation, ALK and ROS-1. Programmed Death Ligand-1 (PD-L1) expression by immunohistochemistry was 70%. The clinical staging with Positron Emission Tomography- Computed Tomography (PET-CT) scan showed mediastinal metastatic lymph nodes and scattered osseous metastases in the axial and proximal appendicular skeleton (Stage IV; T4, N3, M1b). Given the high expression of PD-L1 > 50%, immunotherapy with pembrolizumab was started at (200 mg) intravenously once every three weeks per KEYNOTE-024 protocol []. Subsequent surveillance with CT showed a significant decrease in the size of the primary tumor in the lungs from 8.5 × 5.5 cm to 2.7 × 0.9 cm with a decrease in the size of bone metastases. In addition, patient’s Eastern Cooperative Oncology Group performance status (ECOG) improved from 2 prior to therapy to 0. After week 25 of pembrolizumab, she developed Raynaud’s like symptoms in both hands with mild non-purpuric erythema, pain and paresthesia at the fingertips bilaterally aggravated by cold weather. A thorough history revealed no prior autoimmune disease, recent trauma or similar symptoms in the past. The patient was started on nifedipine 30 mg extended release orally once daily due to potential vasospasm demonstrated by the triad of pallor, cyanosis and hyperemia. There was no improvement of symptoms on 1 week follow up and nifedipine dose could not be increased due to labile blood pressure. The mild intermittent discoloration progressed to persistent periungual blue discoloration over the course of 2 weeks in the digits bilaterally. The patient was started on oral prednisone 30 mg daily (0.5 mg per kg daily) in conjunction with nifedipine. An autoimmune panel revealed anti-nuclear antibodies (ANA) of 1:80 with a homogenous pattern; erythrocyte sedimentation rate (ESR) of 70 mm/hr. Further testing, including ANCA-c, ANCA-p was negative. Complement levels were within normal limits in our lab reference range. Simple nailfold capillaroscopy did not yield any abnormalities. Additional work up did not reveal a septic source, hypercoagulable disease, hyperviscosity related immunoglobulinopathies, lymphoproliferative disorders or vascular occlusion as shown in Table . The patient continued on pembrolizumab given the significant interval tumor response. Over the course of 2 weeks, the patient experienced progression of the discoloration into dry gangrene, ulceration with necrosis involving all fingertips and extending to the proximal phalanges in right digits (1st, 2nd, 3rd, 4th) and (1st, 2nd, 3rd, 5th) digits in the left (Fig. ). Arterial Doppler ultrasound showed normal arterial waveforms through the upper extremity arteries with peak systolic velocity ratios within normal limits. There were no signs of stenosis, atherosclerotic disease or occlusion. CT angiography showed a normal appearance of the arterial branch up to the palmar arch artery without evidence of saccular aneurysm formation, stenosis or occlusion; digital arteries were not adequately visualized. The patient underwent sympathectomy given the rapid progression of necrosis with no response to calcium channel blockers or glucocorticoids. The decision of amputation was deferred until necrotic tissue would have clear demarcation (Table shows course of progression).
Pembrolizumab was stopped later (week 37) due to tumor progression and it was noted that the involved necrotic area in the digits did not progress further after discontinuation of PD-1 inhibitor. Unfortunately, the patient transferred to hospice care and expired two months after. |
pmc-6518657-1 | We report a case of the patient, a 28-year-old woman with a regular 28-day menstrual cycle (FSH level 5,1 U/L), who was diagnosed with carcinoma of the left breast. The overall timeline of the medical procedures from cancer diagnosis to the birth of a child is shown in Fig. .
In cancer diagnosis, the postoperative pathology revealed poorly differentiated invasive ductal high-grade carcinoma, with negative sentinel node biopsy (pT2 pN0 M0 G3 stage IIA by TNM classification) in 2010. The patient underwent targeted sequencing of cancer tissue using TruSight Cancer panel (Illumina, Cat No FC-121-0202) of mutational hotpots in genes predisposing or associated with cancer. Analysis of the targeted regions did not reveal any cancer-related variants/mutations in BRCA1, BRCA2, PTEN, STK11, CDH1, CHEK2, BRIP1, ATM, PALB2, NF1 and TP53 genes.
The option of ovarian tissue cryopreservation was offered as fertility preservation because of the time limitation before chemotherapy, as there was not enough time for hormonal stimulation to produce mature oocytes for freezing. It was also known that the cancer therapy planned for the patient (chemotherapy and radiotherapy) would be highly gonadotoxic and would likely lead to premature ovarian insufficiency and a loss of menstrual cycle activity. The patient granted her written informed consent for conducting the procedure.
During laparoscopy, two ovarian pieces, sized 1.5 × 1.0 cm, were obtained from both sides, containing ovarian cortex and medullar part. The ovarian cortex was manually dissected from medullary tissue in HEPES-buffered IVF culture media (Origio, Denmark). The cortical part of the tissue was cut into smaller pieces (1-2 mm × 3-5 mm) with a thickness of 1-2 mm. For cryopreservation, the cortical slices were incubated with human serum albumin (HSA, 25 mg/ml) for 5 min, 1.5 M 1,2-propanediol (PrOH) for 10 min, and 1.5 M PrOH and 0.1 M sucrose for 5 min in PBS (Sigma-Aldrich, Germany) at room temperature; were transferred to cryovials and were frozen using slow freezing protocol as described previously [].
After the surgery for breast cancer and laparoscopy to remove the ovarian tissue, the patient was eligible for adjuvant chemotherapy and received six cycles of the FEC regimen containing a sequential combination of anthracyclines: three cycles of 5-fluorouracil 500 mg/m2 (800mg), epirubicin 80 mg/m2 (130mg) and cyclophosphamide 500 mg/m2 (dosage 800 mg intravenous). Since the patient developed neutropenia during the chemotherapy, reduced doses of anthracyclines were administered for the last three cycles: 5-fluorouracil (600 mg), epirubicin (110 mg) and cyclophosphamide (dosage 600 mg). The chemotherapy lasted for 5 months in 2010–2011, followed by radiotherapy to the breast tissue (50Gy) and boost to the operation scar (10Gy) for two months in 2011. The patient developed amenorrhea shortly after the initiation of chemotherapy. However, no hormonal tests are available to the authors as they were made in another hospital.
After chemo- and radiotherapy-induced amenorrhea, the patient regained rare spontaneous menstrual cycles at the beginning of 2012. Her FSH level was 22,1 U/L and AMH level was 0,04 ng/mL. Despite of the irregular oligomenorrhea, a spontaneous ectopic pregnancy was diagnosed in the right fallopian tube in the same year. After removing the ectopic pregnancy, the irregular oligomenorrhea lasted until 2013, indicating the deterioration of the ovarian follicular pool after cancer treatment. This was considered as a strong indication for using the ovarian tissue grafting to obtain the pregnancy, when the patient was subsequently counselled for her wish for motherhood.
The decision to use the heterotopic ovarian transplantation was based on the patient’s clinical conditions, what prevented the ovarian tissue orthotopic transplantation. Briefly, recurrent laparoscopies had revealed several pelvic pathologies: before and after cancer therapy, recurrent cystic benign ovarian tumours were found, arising from the left ovary. The tumour completely filled the rectouterine pouch (cavum Douglas) and dislocated the uterus to the right. In addition, mild endometriosis and several abdominal adhesions were diagnosed between the intestines, uterus and tumour tissue. Since the patient had also a spontaneous ectopic pregnancy in the right fallopian tube, likely damaging the tissue, the heterotopic ovarian tissue transplantation was the only option to restore her fertility.
Thawing of five ovarian cortical pieces was performed using the following steps: the cryovials were placed in the 30 °C water bath until the ice was melted. Then the pieces of cortical strips were incubated in 1 M PrOH and 0.2 M sucrose for 5 min and followed by 0.5 M PrOH and 0.2 M sucrose for 5 min in room temperature. Thereafter the pieces were placed into 0.2 M sucrose in HSA (25 mg/ml) in PBS for 10 min. Heterotopic transplantation was used for thawed pieces of ovarian cortex into a submuscular pocket on the left side of the lateral pelvic wall and marked with a metallic marker for better ultrasound visibility (as shown in Fig. ).
After the ovarian tissue transplantation, during the following three months, the FSH level declined to 14,1 U/L and AMH level raised to 0,3 ng/mL. In the middle of 2013, the patient regained her first spontaneous menstrual cycle after tissue grafting.
For controlled ovarian hyperstimulation, we classified the patient as a possible poor ovarian responder, and used the gonadotropin-releasing hormone (GnRH) agonist long protocol with Diphereline (3.75 mg) administration started on the 21st cycle day. On the third day after the beginning of bleeding, the patient started treatment with exogenous recombinant follicle-stimulating hormone (FSH, Gonal-F, 225 IU per day for 2 days, and 300 IU per day from day 3 to 12). The follicular growth and endometrial thickness were measured using ultrasound combined with serum estradiol (E2) measurements to time the injection of human chorionic gonadotropin (hCG) for oocyte meiotic resumption. Recombinant hCG (Ovitrelle, 250 μg) was administered to the patient and the oocyte pickup (OPU) was performed 36 h later.
On the day of OPU, the patient had three follicles of ≥18 mm in the location of the transplanted tissue. Due to the lack of the follicles, the ovaries remained unpunctured and the oocytes were only retrieved from the grafted ovarian tissue, excluding any chance for natural conception. The oocyte retrieval from the transplanted tissue was uncomplicated. All three follicles were aspirated, and two oocytes were obtained. After OPU, the patient was treated with progesterone (Duphaston, 10 mg) by oral administration, until week twelve of gestation. Since the E2 level dropped in mid-luteal-phase, the E2 (Estrofem, 4 mg orally) was also included in the treatment.
Normal IVF was done with both of the oocytes fertilized and one blastocyst was transferred to the uterus on day 5 after oocyte insemination. A positive serum hCG test (593.1 IU/l) performed two weeks after embryo transfer confirmed pregnancy. Subsequently, clinical pregnancy was ascertained in the sixth week of pregnancy, with the gestational sac measured 2.04 cm, crown-rump length 0.49 cm, and heart beating. The timely development of the pregnancy per IVF embryo transfer precluded the natural conception.
At week 28 of pregnancy, gestational diabetes was diagnosed, and although the low carbohydrate diet was recommended for the patient, at week 32, additional Metformin (500 mg twice per day) was administered. The healthy boy (3138 g) was born at 39 + 0 weeks of gestation in April 2014 (Figs. and ).
By the year of 2016, the computed tomography scans had not shown any changes in the breast tissue or formation of metastases. The patient had regained regular 28-day menstrual cycles, for the first time after cancer therapy, indicating the presence of normal ovarian endocrine function. Surprisingly, in 2017, the patient had a spontaneous pregnancy, which could have happened only via recovery of endogenous ovarian function, because the conception via heterotopic transplant would have been possible only using IVF.
At week 20 of the pregnancy, again the gestational diabetes was diagnosed and the patient was recommended low carbohydrate diet. The healthy girl (4100 g) was born at 39 + 0 of gestation in November 2017.
Both of the children have developed normally without major complications. However, both of the children have had some difficulties with the autoimmune diseases such as atopic dermatitis. Also, the first child was diagnosed with juvenile arthritis at the age of 3.5 years. |
pmc-6518661-1 | A 48-year-old woman presented to our outpatient neurology with a headache, nausea, and vomiting for one day. Her headache was persistent, tolerable and blunt in the right temporal region. She had a hypertension history for three years and had been controlled within normal range by antihypertensive drugs. She had no psychosocial and familial hereditary history. Neurological examination was normal. Routine blood tests, serum C-reactive protein level and erythrocyte sedimentation rate showed normally. Serum thyroid hormone and sex hormone level were also in the normal range. Serum anti-MPO and P-ANCA were weakly positive, while other indicators including antinuclear, anti-SSA, anti-SSB, anti-dsDNA, anti-Sm, anti-RNP, anti-Scl-70, anti-RP3, anti-GBM, anti-neutrophil cytoplasmic antibodies in cytoplasmic, antibodies to neuro-paraneoplastic syndromes, neuromyelitis optica (NMO), myelin basic protein (MBP) and myelin oligodendrocyte glycoprotein (MOG) in blood as well as cerebrospinal fluid (CSF) were negative. Serum tumor markers were negative. Lumber puncture showed higher intracranial pressure with 240 mmH2O. Subsequent CSF analysis showed normal protein, glucose, and cell count. Testing on the bacterial, viral, fungal and cryptococcal pathogens of CSF was negative. Magnetic resonance imaging (MRI) of the brain revealed multifocal white matter hyperintensity (WMH) lesions (cortico-subcortical) on T2 and fluid attenuated inversion recovery (FLAIR) associated mass effect, which were slight hypointense on T1 and didn’t enhance on gadopentetate enhanced MRI (Fig. a-d). Susceptibility weighted imaging (SWI) revealed multiple cerebral microbleeds in cortical and subcortical areas (Fig. e). Magnetic resonance spectrum (MRS) demonstrated a normal spectrum. (Fig. f). Magnetic resonance angiography showed normally (Fig. g). Magnetic resonance venogram showed thinness in the bilateral transverse and left sigmoid sinus and decreased signal intensity (Fig. h). Further digital subtraction angiography excluded venous sinus thrombosis. The genetic sequencing showed ApoE ε4 homozygosity and a variant in SORL1 (Fig. a-b).
Based on these findings, we made a diagnosis of probable CAA-ri. We firstly treated the patient with dexamethasone 10 mg/day via venous for three days and then reduced it by half for three days, followed by oral prednisolone 60 mg/day tapered within six weeks (a weekly reduction of 10 mg). On the 3rd day of treatment, her headache was significantly relieved. MRI demonstrated that the lesions on FLAIR almost completely disappeared six weeks later (Fig. a-b). She presented to our clinic with complaints of a headache again after corticosteroid withdrawal for three months. MRI indicated CAA-ri relapsed and SWI showed that the microbleeds in cortical/subcortical areas were increased (Fig. a-b). We launched the methylprednisolone 1000 mg pulse therapy for her. Lesions on T2/FLAIR disappeared quickly. We recommended reducing the dose of corticosteroid gradually until a long-term administration of oral prednisone 5 mg/day. No recurrence was found and the lesions of microbleeds were not increased any more over the next four-month follow-up (Fig. a-b). |
pmc-6518690-1 | Eighty-three years old woman following a fall was put on conservative management at another hospital. Initially her physical examination was mostly normal other than spinal tenderness and she had no neurological (Sensory or motor) deficit. A week later she started having severe back pain and two weeks later she also developed bilateral buttocks pain, bilateral lower limb weakness and had diminished sensation in the sacral area.
Revealed severe tenderness at thoracolumbar junction and neurologic examination demonstrated grade 4/5 strength in the lower extremities. Sacral sensation decreased, bladder and bowel function was intact.
Initial magnetic resonance imaging (MRI) revealed vertebral compression fracture of L1 body without burst and no canal compromise (Figs. a & a). One week later the MRI and computed tomography (CT) revealed compression fracture with intravertebral cleft and beginning of the burst of the vertebral body. A fragment was retropulsed into the canal (Figs. b, b2, and b). An MRI two weeks from initial fall shows aggravation of the burst fracture and thecal sac compression (Figs. c & c). A diagnosis of Kummell’s disease at L1 vertebra with burst fracture of the body with spinal canal compromise and neurological deficit was made.
Polymethylmethacrylate (PMMA) augmented posterior compressive, short segment percutaneous pedicle screw (PA-SSPPF) fixation was done at T12, L1 and L2 vertebrae levels. Postoperatively the height of L1 body was restored and thecal compression disappeared (Figs. d & d). The patient experienced significant pain relief (Preoperative VAS 8 and postoperative VAS 3) and could ambulate with a brace on first postoperative day. Three months later the vertebral body height, stability was well preserved and patient had no neurological deficit (Figs. e & e).
Is a novel concept in minimally invasive spine surgery (MISS). In our case the left pedicle screws of the T12, L1 & L2 were inserted first followed by the right screw of the L1 vertebra. Using the inserter the rod was slide through the pedicle screws on right side. Rod blockers of T12 pedicle screw was tightened first then T12 & L1 pedicle screws compressed and with the help of the compressor placed horizontally a cranially distractive force applied using left L1 pedicle screw as pivot points and the rod blockers of the right L1 pedicle screw tightened. This maneuver is repeated with of L1 and L2 where a caudally directed distractive force was applied using L1 pedicle as pivot. The cumulative result of this maneuver was ligamentotaxis and reduction of the retropulsed fragment Figs. d, d, and a & b). This was followed by insertion of remaining two contralateral pedicle screws and fixation. As described earlier all the screws were PMMA augmented inserted using minimally invasive screw insertion technique. |
pmc-6518705-1 | A 65-year-old male diagnosed with COPD 5 years prior was admitted to our hospital in November 2017. He had stopped smoking 2 years prior, but had a smoking history of 80 pack years. He had been taking indacaterol/glycopyrronium once daily and had been on 3.5 L/min home oxygen therapy for 2 years. In the past year, he had experienced two acute exacerbations that required hospitalization. A pulmonary function test (PFT) conducted in October 2017 revealed severe obstructive lung disease: the ratio of forced expiratory volume in 1 s (FEV1) to forced vital capacity (FVC) was 29%, the FEV1 was 0.41 L (percentage of predicted FEV1, 13%), the residual volume (RV) was 6.43 L (percentage of predicted RV, 275%); the total lung capacity (TLC) was 8.23 L (percentage of predicted TLC, 135%), and the percentage of predicted diffusing capacity of carbon monoxide (DLCO) was 23%. Arterial blood gas analysis revealed a pH of 7.413, PaCO2 of 53.8 mmHg and PaO2 of 65.4 mmHg.
Chest computed tomography (CT) performed in May 2017 indicated severe centrilobular emphysema in both lungs with huge bullae in the right middle lobe (Figs. c and e). The maximum area of the huge bullae in the axial view was 15.0 × 10.1 cm. On CT, the bullae became larger over time and the right lower lobe parenchyma became increasingly compressed. The fissure around the right middle lobe (the target lobe) was intact on chest CT.
We decided to perform BLVR using an unidirectional endobronchial valve. Atropine 0.5 mg was administered 30 min before bronchoscopy to minimize bronchial secretions. To locally anesthetize the oropharynx, we delivered 2 mL of lidocaine using a nebulizer. Commencing with bronchoscopy, we administered midazolam 3 mg for sedation and instilled lidocaine 10 mL for local anesthesia of the vocal cords and large airway. Using the Chartis system (Pulmonx, Inc., Palo Alto, CA), a catheter with a balloon at the distal tip was placed in the entrance of the right middle bronchus. The balloon was inflated to block the airway and the distal airflow, resistance, and pressure were measured. A gradual decrease in flow and increase in resistance and pressure were observed. Thus, we confirmed the absence of collateral ventilation of the right middle lobe []. The right middle bronchus was sufficiently small to be blocked by a single endobronchial valve; for this, we selected a Zephyr 5.5 endobronchial valve (Pulmonx).
After endobronchial valve insertion, chest X-rays were taken for 3 days and 1 week later, and confirmed that the size of the huge bullae had decreased dramatically (Fig. a and b). Two months after valve insertion, we performed chest CT, which showed that the endobronchial valve was located in the right middle bronchus, and that the huge bullae in the right middle lobe had disappeared. The volume of the right middle lobe had thus decreased but that of the compressed right lower lobe had re-expanded (Fig. d and f).
We performed a PFT at 2 months after valve insertion. The ratio of the FEV1 to FVC increased from 29 to 32%, and the FEV1 increased by 170 mL from 0.41 L (percentage of predicted FEV1, 13%) to 0.58 L (19%). The RV decreased from 6.43 L (percentage of predicted RV, 275%) to 4.74 L (201%), and the TLC decreased from 8.23 L (percentage of predicted TLC, 135%) to 6.61 L (108%). The symptoms and quality of life improved markedly, and no valve migration or obstruction, pneumonia, or pneumothorax has been noted to date. |
pmc-6518723-1 | A 63 year old male with renal cell carcinoma underwent nephrectomy in 2015. He was under active surveillance until 2017, when he presented with metastatic disease in lymph nodes and lungs. The patient received nivolumab combined with ipilimumab, a CTLA-4 inhibitor, for 3 months followed by nivolumab monotherapy at a dose of 250 mg every 2 weeks. After 6 months of treatment, he developed immune-related type I diabetes, starting insulin, and continued immunotherapy. After receiving 14 infusions of nivolumab, 253 days after the first infusion, he developed acute left knee pain and swelling and was referred to the rheumatology clinic. Patient denied any prior similar episodes, but had a remote history of injury to his left knee, decades before, with ligament damage, for which he underwent arthroscopy.
Past medical history included hypertension, hypothyroidism and stage 2 chronic kidney disease, all diagnosed prior to immunotherapy. He had no history of recent trauma. One of his children had gout.
Physical exam revealed a large effusion in the left knee with erythema, tenderness, and limited range of motion. Arthrocentesis was performed and synovial fluid analysis showed 6715 white blood cells per μL, 72% of neutrophils, and numerous intracellular and extracellular calcium pyrophosphate dihydrate (CPPD) crystals (Table ). Gram, acid-fast bacilli (AFB), and fungal stains, and cultures were negative. Anti-nuclear antibody, rheumatoid factor, and anti-cyclic citrullinated peptide (CCP) antibody were negative. Knee x-ray showed tricompartmental degenerative changes without fractures or bony metastases. Although very subtle, the x-ray showed chondrocalcinosis of fibrocartilage, hyaline cartilage and suprapatellar bursal synovium. Patient received an intra-articular injection of 40 mg triamcinolone, with an excellent response. Nivolumab was held, and restarted 3 weeks later as the patient had complete resolution of his left knee arthritis. A few days after the infusion, the patient had another flare of left knee pain and swelling. Knee arthrocentesis followed by intra-articular triamcinolone injection was performed with an excellent clinical response within 2 weeks. Synovial fluid again showed CPPD crystals. Two weeks later, after complete resolution, nivolumab was resumed, however, once again, a few days later, he developed left knee arthritis with CCPD crystals in the synovial fluid, and patient received intra-articular triamcinolone. Of note, his thyroid-stimulating hormone (TSH) levels were elevated during the 2nd and 3rd pseudogout flares. Tumor staging showed stable disease, and the patient decided to continue nivolumab therapy despite the recurrent pseudogout flares. He initiated colchicine 0.6 mg orally daily as prophylaxis. After resolution of his knee symptoms, he resumed nivolumab treatment. Patient has continued nivolumab treatment with colchicine prophylaxis and has received three additional nivolumab infusions at the same dose without pseudogout flares. He is now receiving nivolumab 480 mg monthly with good tolerance and only mild pain after the infusions. As of last follow-up he had minimal knee effusion with no pain, tenderness, or limitation in the range of motion. He was able to ambulate and perform activities of daily living. He has had one additional episode of left knee arthritis, but much milder than previous ones, and no arthrocentesis was performed. His magnetic resonance imaging (MRI) of the knee showed primarily partial meniscal tears and damage to his anterior cruciate ligaments. |
pmc-6518727-1 | A 78-year-old man was admitted to a local hospital because of leg swelling and polyuria (both lasting for approximately 2 months). He had not received any medication for his complaint. He had no known medical history of hypertension, diabetes mellitus. Physical examination showed the patient to be well nourished but he craved for water. Retroperitoneal fibrosis was detected on computed tomography (CT). The retroperitoneal fibrotic tissue was compressing both distal ureters, and bilateral hydronephrosis was identified on CT (Fig. a and b). To resolve the hydronephrosis, a double J stent was inserted in the right-side ureter; however, insertion into the left-side ureter failed due to atrophy. To evaluate the cause of the retroperitoneal fibrosis, we decided to perform a laparoscopic surgical biopsy. Because the retroperitoneal fibrotic tissues were too stiff for biopsy, we biopsied the para-aortic lymph node, just adjacent to the fibrotic tissue. The biopsy test results showed the classical characteristics of Castleman’s disease (hyaline-vascular type, negative for human herpesvirus 8).
Before the initiation of radiation treatment, he still complained of frequent urination (20 times/day), excessive thirst (visual analogue scale 8) [], and his urine output was approximately 5~6 L/day. His baseline biochemical levels were as follows: blood urea nitrogen, 19.6 mg/dL; creatinine, 1.4 mg/dL; sodium, 149 mEq/L; potassium, 4.8 mEq/L; chloride, 118 mEq/L; serum osmole, 311 mOsm/kg; and random glucose, 131 mg/dL. On urine analysis, the specific gravity was under 1.005, representing diluted urine, and the urine osmolality was 148 mOsm/kg. No protein, glucose, or red blood cells were seen on a urine analysis. A basal plasma AVP level was 5.24 pg/ml, which was above the normal range (0~4.7 pg/ml). An antinuclear antibody test was performed to further evaluate the retroperitoneal fibrosis. The antinuclear antibody test was positive, with a homogeneous pattern, but the specific tests for extractable nuclear antigen antibodies and double-strand deoxyribonucleic acid were negative.
A water deprivation test was not performed, as his serum osmolality (311 mOsm/kg), and serum sodium (149 mEq/L) were above the threshold for maximal AVP secretion (serum osmolality, 300 mOsm/kg; serum sodium, 145 mEq/L). Thus, we performed a vasopressin challenge test (Additional file : Figure S1). After the vasopressin injection, his urine osmolality increased to 206 mOsm/kg, which was approximately 39% greater than that at baseline (before the vasopressin injection: 148 mOsm/kg). Thus, the urine was not sufficiently concentrated to the expected range, indicating partial nephrogenic DI. Partial nephrogenic DI can be diagnosed as a small elevation (up to 45%) in urine osmolality after a vasopressin injection, with the urine osmolality remaining well below isosmotic urine. Compared to that in patients with partial nephrogenic DI, patients with partial central DI usually achieve a urine osmolality of 300 mOsm/kg or higher after a vasopressin injection [, ]. After the vasopressin challenge test, he still complained with remained thirst (visual analogue scale 4). We treated the patient with hydrochlorothiazide (25 mg/day), and his urine output gradually decreased to within the normal range (Fig. ). After treatment of hydrochlorothiazide, his serum sodium level recovered to between 138 and 142 mEq/L. The patient recovered uneventfully and underwent treatment for Castleman’s disease in our hematology department. |
pmc-6518730-1 | A 15-year-old boy complained of central vision loss in his right eye, which persisted for 2 weeks. The patient’s medical and family histories were unremarkable.
The patient’s best-corrected visual acuity was 1.2 OU with no refractive error. Slit-lamp examination revealed no abnormal findings OU. Funduscopic examination revealed a wedge-shaped dark reddish lesion at the macula OD and no abnormal findings OS (Figs. a and a). Scanning laser ophthalmoscope (SLO) infrared imaging showed a dark area corresponding to the dark reddish lesion (Fig. b). EDI-OCT showed loss or disruption of the interdigitation zone (IZ) at the macula OD and normal findings OS (Figs. e and b). The patient was diagnosed with AMN OD.
On the next day following the first visit, the patient complained of central vision loss OS. One week after the first visit, a wedge-shaped dark reddish lesion was observed at the macula OS (Fig. c, d). EDI-OCT showed the disruption of the IZ at the fovea OS (Fig. e). Fluorescein angiography revealed hypofluorescence from the initial to late phases corresponding to the lesion OU (Fig. c). On indocyanine green angiography, initial geographic hypofluorescence was observed corresponding to the AMN lesion OU (Fig. d), but the finding became obscure at the late phase. Fundus autofluorescence (FAF) revealed faint hypo-autofluorescence, corresponding to the lesion OU. Humphrey threshold 10–2 perimetry showed decreased central sensitivity corresponding to the lesion. The patient was diagnosed with AMN OU and was monitored with no treatment. Three months after the first visit, the dark reddish lesions and a dark area on SLO improved OU. EDI-OCT showed recovery of the macular IZ (Figs. f and f). On FAF, hypo-autofluorescence resolved OU.
Using LSFG-NAVI (Softcare, Fukuoka, Japan) and EDI-OCT (RS-3000 Advance; NIDEK, Gamagori, Japan), central choroidal thickness (CCT) and mean blur rate (MBR) at the macula were sequentially measured.
EDI-OCT measurements were obtained from both eyes at the initial visit, 1 and 3 weeks, and 1 and 3 months after the first visit. CCT was determined by manually measuring the distance between the outer border of the hyper-reflective line corresponding to the retinal pigment epithelium and the outer border of the choroid (Figs. e, f and b, e, f), using a horizontal scan through the fovea (scan length, 12.0 mm). Two authors (Yuki. H., Yuka. H.), blinded to clinical information, independently evaluated EDI-OCT images and the average values of two authors were compared.
LSFG was conducted 5 consecutive times at 1 and 3 weeks and 1 and 3 months. The circles used in obtaining these images were set to the AMN lesion site (Figs. g, h and g, h). The positioning of the circles was decided manually by comparing fundus photography with LSFG color map. The average MBR values were calculated for each phase using the LSFG Analyzer software (v 3.0.47; Softcare, Fukuoka, Japan), which allowed automatic setting of each circle during the follow-up visit at the same site where a circle was previously set at baseline to ensure consistency. Sequential changes in average MBR were evaluated as the changing rates of average MBR against the first measurement values, because MBR is a quantitative index of the “relative” blood flow velocity.
Within a certain range, there is a linear relationship between choroidal blood flow and OPP in healthy subjects [, ]. To exclude the possibility of such physiological responses from the current results, OPP was calculated from the patient’s blood pressure and intraocular pressure, as described previously [].
CCT changes in the present case are shown in Fig. a. In the right eye, CCT decreased from 288.0 μm at the first visit (at the acute stage of AMN) to 272.0, 222.0, 231.0, and 202.0 μm at 1 week, 3 weeks, 1 month, and 3 months later, respectively (Fig. e, f and a). In the left eye, CCT increased from 206.0 μm at the first visit (before the onset of AMN) to 222.0 μm after 1 week (at the acute stage of AMN, Fig. b, e) and decreased to 177.0, 177.0, and 165.0 μm at 3 weeks and 1 and 3 months after the first visit, respectively (Figs. f and a).
The LSFG color map obtained 3 months after baseline clearly demonstrated that the MBR values at the lesion sites were higher than baseline data in both eyes (Fig. b). The average MBR values were as follows: 6.9 ± 1.2, 8.3 ± 0.8, 10.7 ± 0.6, and 9.7 ± 0.6 OD; 6.0 ± 0.4, 9.1 ± 0.6, 10.3 ± 0.6, and 10.1 ± 1.0 OS at baseline, 3 weeks and 1 and 3 months, respectively. When the rate of change in MBR was evaluated using the MBR values at baseline, 20.4, 54.9, and 40.0% increments were noted OD at 3 weeks, 1 month, and 3 months, respectively. Similarly, 51.1, 70.6, and 68.3% increments were noted OS at 3 weeks, 1 month, and 3 months, respectively. OPP slightly decreased from 31.0 mmHg OD and 34.0 mmHg OS at baseline to 26.4 mmHg OD and 27.4 mmHg OS at 3 months. |
pmc-6518768-1 | A 70-year-old Chinese man was presented in the study. The patient had the past medical history of diabetes, hypertension and cerebral infarction, and had received hemodialysis three times a week due to renal failure for 2 years. SHPT was confirmed in December 2016 since the serum PTH had elevated 100.3 pmol/l(normal range 1.6–6.9 pmol/l). Cinacalcet(at a dose of 25 mg per day) was prescribed based on the concurrent hypercalcemia and hyperphosphatemia. In April 2017, the PTH level was stable at 94.44 pmol/l due to his poor compliance, with normal calcium level(2.06 mmol/). Subsequently, the patient was given calcitriol pulse therapy(at a dose of 2μg for trice weekly) combined with low calcium dialysate(Ca2+, 1.25 mmol/l). Unfortunately, the PTH level bounced high up to 197.3 pmol/l dramatically. Since August 2017, the patient was constantly afflicted with the uncomfortable and irritating sensation in the legs urging him to walk or move his legs. Even worse, the patient had more complaints, such as loss of appetite, nausea, constipation, refractory hypertension, generalized weakness and sleep deprivation. Otherwise, he had an normal physical examination. Laboratory test revealed that serum albumin corrected calcium jumped up to 3.96 mmol/l and the alkaline phosphatase(ALP) level was 160u/l(normal range 45-125u/l), supporting the diagnosis of hypercalcemic crisis and tertiary HPT.
Moreover, several examinations were also completed to identify any possibilities. However, the patient had no bone pain or facture, and no abnormality was revealed in bone X-ray imaging. Besides, M protein and tumor markers were negative as well. Surprisingly, no abnormality was found in parathyroid ultrasound around neck. Given the threat of hypercalcemia crises, continuous renal replacement therapy(CRRT) with low calcium dialysate(Ca2+ 1.25 mmol/l) was applied instantly. Meanwhile, calcitriol pulse therapy was ceased while higher-dose cinacalcet(50 mg per day) was reused again. In addition, calcitonin was subcutaneously injected at a dose of 50u per day to lower the blood calcium, but poor response was achieved, and the serum albumin corrected calcium kept elevating to 3.77 mmol/l. Therefore, hormone therapy(intravenous injection of 40 mg methylprednisolone for 7 day, and oral administration of 20 mg prednisone until operation) was considered.
To our attention, the bone turnover makers, such as osteocalcin and I-type collagen prolongation peptide, had surpassed the upper normal limit(300 ng/ml and 1200 ng/ml, respectively), while the β-crosslaps was 5.96 ng/ml. In addition, the high bone turnover and remodeling rate, as well as the refractory HPT, had pointed to the presence of an ectopic parathyroid gland. Thus, the patient was further examined through computed tomography (CT). As expected, a contrast-enhanced neck CT scan had revealed a 2.5*1.9 cm mass in the upper region of anterior mediastinum, which had displayed nodular mixed density (Fig. a, b). More importantly, the Tc-99 m dual-phases parathyroid scan had detected a hyperfunctioning parathyroid gland in the same region(Fig. c). Consequently, it was concluded that the mass in the anterior mediastinum might be the ectopic parathyroid gland.
Despite of high anesthesia risk caused by hypocalcaemia, thoracic aortic calcification, and adherence to aortic arch, the mass was completely excised through video-assisted thoracoscopy (Fig. d). Biopsy was performed, and histopathological examination revealed a 2 cm well-encapsulated mass containing solid and cystic components. In addition, clear and transplant liquid could be observed inside the cyst, and a gray solid nodule had adhere to the wall. Microscope observation suggested that, the tumor was composed of the parathyroid gland chief cells, especially in the nodule.
Therefore, the calcium and PTH level had dropped stably within 10 days after surgery(2.12 mmol/l and 21.41 pmol/l, respectively), and they had been maintained for 3 months after surgery (2.30 mmol/l and 18.63 pmol/l, respectively). Besides, the phosphorus level(1.77 mmol/l) has dropped strikingly, while ALP bounced to 347u/l. The patient was then free from the restless legs syndrome and gastrointestinal distress. So far, he has been followed up for 16 months, and complications, including hungry bone syndrome, hypocalcemia, hemorrhage and infection, are not observed. On February 14th in 2019, the calcium, phosphorus, ALP and PTH were retested to be 2.42 mmol/l, 1.83 mmol/l, 70u/l and 13.77 pmol/l, respectively. Bone turnover had returned to normal level, since the levels of osteocalcin(121.3 ng/ml), I-type collagen prolongation peptide(213.5 ng/ml) and β-crosslaps (0.707 ng/ml) were reduced. |
pmc-6518787-1 | A 35-year-old Chinese man presented at our hospital with pain and limited movement in his right hip for the last 6 months. Although he had previously received medical treatment and joint-puncture treatment at local hospitals, there had been no improvement in his condition. His case history indicated that he had worked in wool processing 6 months before presenting at our hospital. We, therefore, suspected that the patient might have acquired Brucella infection through inhalation or even contact with injured skin. At the time of admission, the patient showed limited active and passive hip movement because of significant pain. His erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) level were 108 mm/h and 36 mg/L, respectively. Remarkably, his pelvic X-ray and computed tomography (CT) findings revealed osteonecrosis of the femoral head, subchondral erosion, and sclerosis (Fig. a–c). Magnetic resonance imaging (MRI) findings of his right hip (Fig. d) showed marked joint effusion and synovitis. We deduced that septic osteoarthritis could explain these clinical and radiological findings. The results of real-time PCR demonstrated the presence of Brucella melitensis in synovial fluid. During hospitalization, the patient was administered doxycycline and rifampicin for 6 weeks. By the time of discharge, his joint pain had resolved completely, and his joint mobility had increased greatly. The patient could walk normally with a walking aid. |
pmc-6518787-2 | A 41-year-old Chinese man presented at our hospital with pain and limited movement in his right hip for the last 2 months. He had no relevant medical history or any history of injury or alcohol/steroid use. He had not received any medical treatment for his complaints. His case history indicated that he had worked in a slaughterhouse for 3 months. We, therefore, suspected that he might have acquired Brucella infection through ingestion or contact with injured skin. At the time of admission, the patient showed limited active and passive movement of the right hip because of significant pain. His ESR and CRP level were 80 mm/h and 29 mg/L, respectively. Pelvic X-ray and CT findings revealed bone destruction, subchondral erosion, and sclerosis (Fig. a–b), while MR images of his right hip (Fig. c–d) showed marked joint effusion, synovitis, and soft-tissue swelling. We again deduced that these clinical and radiological findings could be explained by septic osteoarthritis. The results of PCR analysis revealed the presence of B. melitensis in synovial fluid. During hospitalization, the patient was administered doxycycline and rifampicin for 6 weeks. At the time of discharge, his joint pain and swelling had resolved completely, and his joint mobility had returned to normal. The patient could walk normally without any aid. |
pmc-6519298-1 | Patient consent for publication was obtained. A 17-year old female presented to an outside hospital (OSH) after she fell down the stairs in her home. She attributed the fall to a 1-month history of numbness, tingling, and weakness in her bilateral lower extremities (BLE), back pain, as well as the sudden onset of blurry vision. On physical exam, she was found to be areflexic in both legs. An initial non-contrast MRI of the brain shortly after admission () revealed a subcentimeter focus of subcortical and cortical T2 hyperintensity in the right paramedian motor cortex. This lesion faintly peripherally enhanced (). This was initially thought to be unrelated to her symptoms. A contrast-enhanced MRI of the spine was unremarkable at this time (). The patient was diagnosed with Guillain-Barre Syndrome and treated with high-dose steroids. Her symptoms did not improve, and the treatment regimen was switched to intravenous immunoglobulin (IVIG). She completed 5 days of IVIG with minimal improvement and was discharged to an inpatient rehabilitation facility. Unfortunately, the patient did not respond to physical therapy (PT), and, in the interim, developed daily headaches associated with photophobia, phonophobia, diplopia, and nausea. No anti-neoplastic treatment was provided. During her PT, she presented to an OSH Emergency Room (ER) with new right arm weakness and slurred speech. Contrast-enhanced MRIs of the brain revealed the progression of her right paramedian motor cortex lesions which had increased in size with solid central enhancement seen of the larger lesion (). An MRI of the spine with contrast was performed demonstrating numerous new, subcentimeter enhancing osseous foci throughout her spine (). A CT of the neck, chest, abdomen, and pelvis and a thyroid US did not identify a potential site for primary disease. An F-18 FDG PET/CT scan performed at this time revealed that a hypermetabolic sclerotic lesion in the left iliac bone and a smaller sclerotic lesion in the right iliac bone without definite hypermetabolism ().
A biopsy of a 0.5 cm right iliac lesion was attempted and demonstrated non-specific atypical cells that stained CD56+ on immunohistochemistry (IHC). The patient then underwent three lumbar punctures (LPs) with high opening pressures and elevated glucose but with negative cytology. Due to her high intracranial pressure, a brain biopsy was deferred at this time.
Two months later, the patient again presented to an emergency department following a generalized tonic-clonic seizure. A repeat LP revealed an increased opening pressure of 60 mm Hg and an MRI of the brain demonstrated the continued progression of her existing lesions in the parasagittal right frontal and parietal lobes with associated increasing infiltrative T2 signal. An MRI of the spine (see ) showed the progression of disease with an increase in size and number of multifocal enhancing osseous lesions of the spine (), MRI of the pelvis also showed new enhancing lesions. F18-FDG PET/CT was repeated now demonstrating FDG avidity of the increasing osseous lesions. All imaging was suggestive of a multifocal, neoplastic process but a slowly progressive atypical infection was not excluded.
Stereotactic biopsy of the right frontal lobe lesion was performed revealing an infiltrative, diffuse high-grade glioma with diffuse strong expression of p53. An H3F3A G34R mutation of specimen tissue was identified by pyrosequencing analysis. A bone marrow (BM) biopsy from the left anterior iliac crest was performed ~2 weeks following the brain biopsy. Evaluation of the bone marrow specimen revealed diffusely metastatic tumor with metastatic cells representing 30% of marrow cellularity within the sample. The immunohistochemical evaluation of the malignant cells revealed a diffuse strong expression for p53, ATRX expression, and focal weak expression for CD56. The profile of the malignant cells in the bone marrow were thought to be consistent with the recently diagnosed infiltrative brain glioma when compared together.
During this admission the patient also underwent a lumbar puncture. Cerebrospinal fluid analysis revealed rare atypical cells thought to be compatible with the glioma. A CT of the chest with contrast performed also at this admission revealed the interval development of a large soft tissue mass in the right lower lateral chest wall centered around a rib (). This was not present on the PET/CT study 9 months earlier. This mass was contiguous along the chest wall with underlying nodular pleural soft tissue lesions and a plural effusion. The patient underwent bilateral pleurocentesis with rare cells in the fluid staining positive for glial fibrillary acidic protein (GFAP).
The patient's metastatic disease continued to progress over the next 2 months with an CT of the abdomen and pelvis with contrast ~2 months after the last admission revealing diffuse osseous metastases throughout the bony pelvis and bilateral proximal femora (). Extensive internal and external iliac adenopathy with encasement and compression of the iliac veins and inferior vena cava was also noted. As part of treatment planning, the dominant mass in the left pelvis underwent a CT-guided biopsy. Pathologic evaluation of the sample revealed malignant cells morphologically similar to the brain biopsy that stained positive for positive for GFAP and S100 protein. |
pmc-6519491-1 | A 78-year-old male came to Scientific Institute Hospital “Casa Sollievo della Sofferenza” for diffuse abdominal pain. His past medical history revealed a total excision of a dorsal cMM 4 years before with no evidence of metastatic disease at the time of diagnosis. Physical examination of the patient appeared to be good in terms of general health and nutritional status: abdomen was soft, with no palpable masses. There was no evidence of melaena and laboratory results were normal. The hepatobiliary ultrasound evidenced multiple intracholecystic nodules involving the gallbladder fundus, body and neck; the parietal lesions appeared like hyperechoic masses of variable sizes (≥1 cm), with minimal to absent acoustic shadowing. The rest of abdomen was poorly explorable due to abdominal adiposity (). Contrast-enhanced CT (CECT) of the abdomen confirmed the gallbladder parietal lesions and revealed multiple solid masses (up to 5 cm) with early contrast-enhancement (CE) and progressive washout located in the stomach, duodenum and ileum. Diffuse peritoneal nodules, mesenteric lymph nodes and pelvic ascites were detected, too (). Chest CT scan showed bilateral solid pulmonary nodules with no pleural effusion (). The possibility of metastatic lesions was considered. Esophagogastroduodenoscopy described multiple, friable, parietal masses of stomach and duodenum with melanosis. Histopathological and immunohistochemical analyses confirmed metastatic melanoma in bioptic material and the patient underwent palliative treatment. |
pmc-6519492-1 | A 30-year-old male patient was examined in our outpatient clinic with a 3-year
history of mild low back pain. In the last 20 days, the pain worsened severely, with
irradiation to the posterolateral aspect of the lower limbs and difficulty to walk.
Palpation of the lumbar spine sacroiliac joints was painful (positive
Patrick’s test). Laboratory analysis showed normal levels of inflammatory
markers (normal reactive C protein).
The patient was a heavy smoker, with no other comorbidities. Initial MRI demonstrated
an infiltrative mass, affecting the vertebral body and pedicles of L4, with
extension to the vertebral canal, with heterogeneous enhancement after contrast
administration (). Tumor extended
into the distal third of the inferior vena cava and the left iliopsoas muscle.
CT scan was also performed (). There
was a lytic lesion with indistinct borders, in the vertebral body of the fourth
lumbar vertebra (L4), associated with a soft tissue component that dislocated the
aorta and the inferior vena cava. There was a typical “ring-and-arc”
chondroid matrix mineralization in the vertebral body of L4 ().
The patient underwent a needle transpedicular biopsy of the vertebral lesion and a
malignant infiltrative bone neoplasm was diagnosed, with chondroid differentiation
and several areas of necrosis.
Three weeks after the needle biopsy, a posterior lumbar approach was performed, with
installation of pedicle screws at L3 and L5 and the posterior elements were removed.
The anterior large vessels were dissected from the spine and an “en
bloc” resection of L4, reconstruction of the lumbar spine with a titanium
cage and antero lateral plate were done.
Histopathological examination of the resected L4 vertebral body revealed an
infiltrative malignant biphasic neoplasia composed of ample ill-defined areas of
atypical cartilage with well to moderate differentiation intercepted by a variable
quantity of poorly undifferentiated small cells with prominent capillary
vasculature, exhibiting hemangiopericytoma-like features (). Involvement of adjacent soft tissues was
noted and the surgical margins were affected. The tumor showed immunoreactivity for
CD99/MIC2 protein on immature cells (); S-100 protein was expressed in cartilaginous areas and SOX-9 was
positive in both components, but mainly in primitive mesenchymal cells (). No expression was found for
epithelial markers (CKs; EMA) and for lymphocytic neoplastic elements. These
morphological findings and immunophenotype of the tumor cells are consistent with
mesenchymal chondrosarcoma (high histological grade).
Patient was discharged after 10 days of the main procedure with controlled pain and
able to walk without assistance. He was seen in our outpatient facility after 20
days with moderate back pain and normal neurological examination. About 2 months
after the spinal reconstruction, he developed a progressive bladder incontinence,
bilateral leg weakness and severe back pain. A new MRI was performed () confirming progression of the disease.
Additionally, a right occipital scalp lesion was visualized and biopsied, evidencing
malignant immature cell neoplasm with chondroid areas, occupying the dermis and
hypodermis, compatible with cutaneous metastasis (). He received local radiotherapy, systemic chemotherapy, and
was able to walk with sticks after 2 months, with mild back pain.
After 5 months of hospital discharge, the patient underwent PET-CT, which showed
progression of the disease, characterized by metastatic implants in the skin and
subcutaneous (occipital and below the angle of the mandible) muscles (serratus
anterior, gluteus maximus and semitendinosus), in bones (sternum, ulna and femur)
and in liver. Thus, palliative treatment was indicated with radiotherapy. |
pmc-6519495-1 | The patient is a 43-year-old female who developed end stage renal disease following an ANCA positive rapid progressive glomerulonephritis, for which she received a living donor renal transplant six years ago. The donor tissue was negative for Epstein Barr Virus (EBV serologies, while the recipient was positive for EBV IgG Antibodies only. She was managed on chronic immunosuppression with a combination of azathioprine and prednisone.
The patient presented initially after an exacerbation of chronic lower back pain following a non-traumatic injury while dancing. Radiographs and MRI of the lumbar spine were performed as part of the initial work-up. Radiographs were negative for acute injury. MRI of her L-spine incompletely captured irregular marrow signal at her left ischium and right sacral ala. This raised the concern for a primary or metastatic marrow replacing process and prompted further imaging work-up. MRI of the pelvis and sacrum performed at this time demonstrated multiple T
2-hyperintense and enhancing osseous lesions involving the lumbar spine, sacrum, pelvis, and proximal left femur. Several lesions demonstrated aggressive features, including periosteal reaction and large extra osseous soft tissue components (). The multifocal nature of the bone involvement and the presence of lytic and aggressive features raised concern for osseous metastatic disease from a distant primary.
CT of the chest, abdomen, and pelvis was performed at this time in attempt to locate a primary site of disease. The CT scan again demonstrated multiple bone lesions with areas of marked cortical destruction (). Numerous round pulmonary nodules measuring up to 1.8 cm, as well as a few enlarged axillary lymph nodes, the largest measuring 2.1 cm in the short axis, were also noted. However, there was no conclusive primary site of malignancy. Fluo-D-glucose positron emission tomography revealed markedly fludeoxyglucose (FDG) avid bone lesions with maximum standardized uptake value up to 35.8 (). Additionally, the rounded pulmonary nodules and prominent thoracic lymph nodes were found to be FDG avid as well, with the SUVmax of the nodules and lymph nodes measuring 23.6 and 30.9, respectively ().
CT guidance was utilized in the biopsy of the large destructive sacral bone lesion. Pathological analysis revealed an EBV negative, diffuse large b-cell lymphoma with a high proliferation rate. confirming the diagnosis of PTLD. The additional thoracic findings were then presumed to be from PTLD given their rounded, mass-like appearance and marked FDG, and therefore biopsy of these lesions was not pursued. |
pmc-6519497-1 | A 62-year-old male with a history of diabetes mellitus and rheumatoid arthritis,
treated with leflunomide and rituximab, presented with bacteremia and acute onset
right knee pain. Physical examination revealed right knee and proximal tibia
tenderness and erythema. Initial laboratory work-up revealed gram-positive cocci
bacteremia, elevated C-reactive protein, and right knee joint aspiration with no
organisms seen on gram stain. |
pmc-6519506-1 | A 41-year-old female with complaints for right-sided upper abdominal pain was admitted to the A.V. Vishnevsky National Medical Research Center of Surgery for diagnostic evaluation and treatment. Ultrasound examination at her local institution had visualized a right liver lobe mass. Biochemical blood assay revealed increased levels of hepatic transaminases. |
pmc-6519508-1 | A fit and well 39-year-old female self-presented to the emergency department with a
2-day history of right-sided flank pain radiating to the back, colicky in nature.
This was associated with frank haematuria. The medical history and examination were
unremarkable with no known comorbidities. Furthermore, the patient was not on any
anti coagulants or anti platelets and there was no history of trauma. |
pmc-6519976-1 | The patient is a 75-year-old female with a past medical history of chronic obstructive pulmonary disease, type 2 diabetes mellitus, atrial fibrillation not on anticoagulation. She had multiple hospital admission due to urinary tract infection and left hip pain due to fracture status post open reduction and internal fixation three months before that presented to the emergency department with acute on chronic left hip pain. Patient’s pain started two weeks ago, and she described it as severe, sharp, constant, radiated to the left thigh, that has been progressively worse to the point that she has been unable to walk; nothing had made the pain better. On exam, she had an externally rotated left lower extremity, tender, cold to palpation, along with a 3-cm decubitus ulcer around the heel.
Initial laboratory workup showed elevated potassium of 7.2, creatinine at 13.34 mg/dL, blood urea nitrogen of 103, initial white blood cell counts 51,200/mcl, platelet 585, lactic acid 7.2, erythrocyte sedimentation rate 97 and C-reactive protein 23. Her electrocardiogram was significant for generalized hyperacute T wave. Given electrolytes abnormalities, a non-tunneled central line catheter was placed for urgent hemodialysis. Before this was able to be done the patient became hypotensive and tachycardic, not responsive to aggressive intravenous fluids resuscitation, requiring initiation of vasopressors. She was taken to the intensive care unit and started on vancomycin and piperacillin-tazobactam for empiric coverage. Additional tests result included a positive urine culture for Escherichia coli along with blood cultures showing Gram-positive rods.
At the time of admission, the primary diagnosis was septic shock secondary to urinary tract infection with suspected blood culture contamination. Further workup included left hip arthroscopy to identify another source of infection due to severe persistent left hip pain and lack of improvement in her overall clinical status, but it was unable to yield synovial fluid. Repeated blood cultures showed no growth for which the urinary tract was suspected to be the primary source of infection. Antibiotics were tailored based on susceptibilities of the urine culture and were changed to Ceftriaxone (Table ). Nonetheless, her admission was complicated by acute hypoxic respiratory failure requiring mechanical ventilation, as well as the addition of multiple vasopressors.
It was not until the third day of hospitalization when the initial blood cultures finalized as Clostridium perfringens (Table ). Due to the lack of improvement and recent blood cultures results, antibiotics were changed again to piperacillin-tazobactam, and the infectious disease specialist was consulted. Recommendations were made to add clindamycin, considering computed tomography (CT) of the hip/pelvis and lumbar spine. We decided to wait for the final susceptibilities report to discuss surgical exploration of the left hip since it was unable to be determined this as the source of primary infection. Despite the latest measurements, the patient continued getting hypotensive and progressed to multiorgan failure resulting in death by the end of the third day. |
pmc-6519979-1 | A 58-year-old woman presented with the first episode of a generalized tonic-clonic seizure. On examination, she had findings of mild left pyramidal hemiparesis associated with upper motor neuron signs. Brain resonance imaging (MRI) studies revealed a 16-mm right parafalcine,extra-axial lesion with homogenous contrast enhancement. There was surrounding vasogenic edema in the adjacent parietal and posterior frontal white matter (Figures -). The lesion was thought to be extra-axial with a narrow dural base (Figure ).
Initial body computed tomography (CT) revealed pretracheal and precarinal lymphadenopathy, although there was no definite primary lesion elsewhere. There was a remote history of a benign breast lesion. Provisional diagnosis favored a meningioma, although a metastasis remained in the differential, given the volume of vasogenic edema and mediastinal lymphadenopathy.
The patient underwent a parasagittal craniotomy and Simpson grade-two excision of the tumor. A small component of residual tumor was left deliberately, as it was adherent to the wall of the superior sagittal sinus. She recovered well following surgery, without any neurological deficits.
A histological examination revealed that the neoplastic cells had moderately pleomorphic nuclei, predominantly spindle in outline, with occasional plump and polygonal forms and eosinophilic cytoplasm. The cells were arranged in a fascicular pattern by variable amounts of myxoid stroma with a low mitotic rate (4/10 high power fields (HPFs)) but necrosis was absent. A well-developed and prominent peripheral lymphoid cuff was present. Immunohistochemically, the tumor cells were positive for desmin, anaplastic lymphoma kinase 1 (ALK1), vimentin, and epithelial membrane antigen (EMA).
Based on these findings, no final diagnosis was suggested. The differential diagnosis was reported between an atypical meningioma and an inflammatory myofibroblastic tumor (IMT). In view of the difficulties resolving the differential diagnosis, the case was referred for international consultation. The outcome was that the lesion had meningioma characteristics.
Due to the abnormal progression of the tumor and uncertainty about the diagnosis, the case was revisited and a fluorescence in situ hybridization (FISH) analysis was performed. The analysis confirmed the disruption of the EWSR1 gene at 22q12 and the disruption of the CREB1 gene at 2q33, without disruption of the ATF gene at 12q13. Based on these cytogenetic findings, in conjunction with histomorphology and immunophenotype, a final diagnosis of a myxoid variant of AFH was confirmed.
A follow-up MRI scan at three months revealed a small area of enhancement along the superior sagittal sinus, consistent with the intraoperative observation of residual tumor (Figure ). The prior vasogenic edema had resolved, with no evident area of cortical thickening and white matter signal abnormality in the right middle frontal gyrus. Clinically, the patient remained asymptomatic since surgery and she reported no further seizure episodes. Subsequent positron emission tomography (PET) scan did not show active distant metastatic disease or fluoro-D-glucose (FDG) avidity in the mediastinal lymph nodes.
Findings on repeat CT scan of the whole body remained stable, including the pretracheal lymphadenopathy. No new lesions were identified on a follow-up PET scan. The recurrent intracranial lesion remained relatively stable on serial MRIs. The option of radiosurgery was discussed with the patient. She had elected to undergo radiosurgery treatment if subsequent MRI scans had demonstrated further progression (Figures -). |
pmc-6519981-1 | Our patient was a 70-year-old female with past medical history significant for non-melanomatous skin cancers and 40 pack-year of smoking who initially presented with complaints of cough and fatigue and was treated with antibiotics for pneumonia. Interval X-ray after non-response to antibiotics showed a right perihilar mass and right middle lobe collapse. Staging computed tomography (CT) of the chest elaborated a 6.5 cm right perihilar mass encasing and obstructing the right middle lobe. Positron emission tomography (PET)/CT and bronchoscopic sampling were undertaken and K-ras positive, PDL-1 unknown adenocarcinoma with involvement of lymph nodes 4R and 7 were confirmed without evidence of distant spread. Imaging had suggested possible invasion of her atrium; however, an echocardiogram did not confirm this finding. Given her Stage IIIB T3 N2 M0 (per AJCC 8th edition) disease, she was offered curative-intent treatment with definitive conventional chemoradiation on a systemic therapy trial randomizing the addition of veliparib, a poly ADP-ribose polymerase (PARP) inhibitor, to conventional carboplatin/paclitaxel during radiation and additionally as part of consolidation. She was treated with 60 Gy using a volumetric modulated arc therapy/intensity modulated radiotherapy (VMAT/IMRT) technique and developed transient esophagitis. Chemotherapy was discontinued during treatment due to poor tolerance; she was taken off the clinical trial. Two weeks after completing radiation treatment, CT imaging demonstrated decrease in size of perihilar primary and mediastinal lymph nodes. However, a left adrenal mass was appreciated measuring 2.8 cm x 2.4 cm (Figure ).
She was started on nivolumab and after receiving two doses, approximately six weeks after completing radiation treatment, she was admitted for complaints of shortness of breath, hypoxemia, and acute right-sided rib pain, and was managed with antibiotics for presumed post-obstructive pneumonia on the basis of radiologic findings of increased parenchymal lung patchy opacities and ground glass attenuation distal to the primary on CT. The right perihilar primary appeared stable in size. CT imaging measured the left adrenal mass as appearing larger at 3 cm x 4 cm (Figure ).
Interval CT scan three months after completing treatment showed slight decrease in size of the perihilar primary and decrease in size of mediastinal lymph nodes. However, at this point bilateral adrenal involvement was noted (Figure -). The previously known left adrenal metastasis appeared enlarged measuring 6.4 cm x 3.7 cm and the newly diagnosed right adrenal metastasis measured 3.2 cm x 2.7 cm. In addition, a newly developed 2.1 cm x 2.1 cm left para-aortic lymph node was disclosed which appeared centrally necrotic (Figure , ).
Interval CT scan two months later (five months since initial definitive treatment) showed stable size in the lung primary with associated obstructive atelectasis and continued significant increase in size of bilateral adrenal masses (Figure -). The left adrenal metastasis measured 8.0 cm x 3.9 cm and appeared heterogeneous; the right adrenal metastasis measured 4.7 cm x 6.5 cm. The left para-aortic lymph node was also noted to have enlarged to 2.5 cm x 2.5 cm (Figure , ).
She subsequently received photon stereotactic body radiation therapy (SBRT) to both adrenal lesions to 25 Gy in five fractions (Figure , ) eight months after initial definitive treatment. Retrospective review of the radiation treatment plan shows the left para-aortic lymph node was essentially included in the left adrenal target volume and hence would have received prescription dose. At the time of treatment the left adrenal mass measured 6.9 cm x 3.5 cm, the right adrenal mass measured 6.1 cm x 5.6 cm, and the left para-aortic lymph node measured 2.5 cm x 2.4 cm. CT scan one month after SBRT (nine months after initial definitive treatment) showed the stable lung primary. Decrease in size of both adrenal masses was noted (Figure -). The left adrenal mass measured 4.4 cm x 2.3 cm and the right adrenal mass measured 2.0 cm x 6.2 cm. The left para-aortic lymph node decreased in size to 1.4 cm (Figure , ).
Interval CT scan three months after SBRT (11 months after initial definitive treatment) demonstrated stable right hilar mass, stable to decreased size of bilateral adrenal masses (Left: 4.4 cm x 2.6 cm, Right: 4.7 cm x 2.9 cm) and continued decrease in size of left para-aortic lymph node (0.9 cm x 0.8 cm) (Figure ). There was noted development of irregular nodular opacities at the medial right lung base measuring 12 mm and 10 mm.
Roughly five months after SBRT (13 months since initial definitive treatment) she was admitted with pneumonia which was thought to be post-obstructive. CT imaging without contrast enhancement at this time measured increase in size of bilateral adrenal masses (Left: 4.9 cm x 3.1 cm; Right: 6.2 cm x 3.5 cm). Right perihilar primary was assessed to have enlarged to 2.7 cm x 3.4 cm from 1.9 cm x 2.6 cm with additional note of increase in size of a right lower lobe nodule from 10 mm to 12 mm and increased coalescence of bandline consolidation in the right hilum along the right lower lobe with surrounding interlobular septal thickening. Nivolumab was held. She was discharged and prescribed a long course of antibiotics. She was noted to have slow recovery in performance status with ongoing anorexia and weight loss. Pulmonary stenting was considered and not pursued. An additional dose of nivolumab was given. Follow-up contrast enhanced CT imaging corresponding to six months after SBRT (14 months since initial definitive treatment) demonstrated progressive findings of enlargement of the bilateral adrenal metastases (Left: 6.0 cm x 3.0 cm; Right: 6.4 cm x 3.5 cm) (Figure , ) with the right adrenal mass demonstrating new invasion of the right hepatic lobe, upper pole of the right kidney, and direct invasion of the inferior vena cava (IVC). New occlusive thrombus in the IVC from the iliac confluence to the level of the renal veins approximately 12.5 cm in length was noted. Two months after her admission, she was noted to have continued poor performance status, increased work of breathing, poor appetite, and ongoing weight loss in addition to tachycardia and hypotension. She was transferred to home hospice where she eventually passed.
Figure graphically summarizes the aforedescribed serial time course changes in bilateral adrenal masses (Figure ) and left para-aortic lymph node (Figure ). |
pmc-6520290-1 | We report a case of 31-year-old right-handed male patient. He came to our neurorehabilitative care unit after hemorrhagic cerebral event. He presented a severe medical history. He was submitted to craniotomy for decompression of hematoma in fronto-parietal right regions. He also presented devices for nutrition (PEG) and for spontaneous breath (Tracheal Cannula). Medical management included Baclofen, Ramipril and Levodopa/benserazide cloridrato. All these clinical aspects were compatible with MMD During hospitalization, the patient in our institute, performed a specific motor, logopedic, neuropsychological rehabilitative treatments.
At first, we immediately conducted a neuropsychological and clinical evaluation. Patient was evaluated as a Vegetative State (VS). For this reason, it was impossible to undertake a global neuropsychological evaluation. Patient was awake, but verbal and nonverbal communication were absent. There was no contact with external environment. We used clinical scales to evaluate evolutions of consciousness's state such as: Coma Recovery Scale-Revised (CRS-R), Level of Cognitive Functioning (LCF), Disability Rating Scale (DRS), and Glasgow Coma Scale (GCS). He was re-evaluated at least 1–2 times/week, to monitor conscious improvements (Table ).
Patient was submitted to magnetic resonance angiography that showed several parenchymal supra- and subtentorial brain arteriovenous malformation (AVMs) with "smoke cloud" evident in different cerebral areas: bilateral cerebellum, occipital and posterior temporal areas, and hippocampus. Magnetic Resonance Imaging (MRI) showed intraparenchymal hemorrhagic lesion in right capsular, fronto-parietal and insular lobe (Figure ). Electroencephalographic (EEG) evaluation was performed at baseline, and after 4 months: it showed altered electrical brain activity due to the presence of slow polymorphous waves (theta and delta rhythms) prevalent in left hemisphere. In addition, we performed Laser-Evoked Potentials (LEPs) examination to evaluate sensory and nociceptive system (De Salvo, Caminiti et al., ; De Salvo, Naro et al., ) and Event-Related Potentials (ERPs) assessment to explore residual cortical cognitive functions (De Salvo, Caminiti et al., ; De Salvo, Naro et al., ).
LEPs examination, at baseline time point (T0), showed an increase of latency (300–440 msec) and an amplitude reduction (8,7 µv) of vertex-complex N2/P2, (more marked in lower limbs), respect to standard value of LEPs (latency: 244–370 msec; amplitude: 16 µv). At follow-up evaluation (T1), the LEPs highlighted a mild improvement of vertex-complex N2/P2 to left lower limb.
We used Neurowave System to perform multisensory stimulation and analyze biophysical parameters (De Salvo, Caminiti et al., ; De Salvo, Naro et al., ). The ERPs recorded, showed moderate improvement between T0–T1. At T0, the P300 wave was absent; while at T1, the P300 wave was present even if the latency and amplitude parameters were significantly altered.
The multidisciplinary team assessed the patient everyday: he was treated for logopedic, physiotherapeutic, and neuropsychological rehabilitation. Physiatrist, in collaboration with neurologists, neuropsychologists, and therapists (logopedists and physiotherapists), planned a rehabilitation program. Members of the patient's family were updated about clinical conditions and the progress of the rehabilitation program every month.
We started rehabilitative treatment 1 month after acute event and was carried out for 6 months (six times a week). Patient was treated two times daily for 60 min to each session. The logopedists, in particular, contributed to tracheostomic cannula with respiratory and phonatory exercises. Physiotherapeutic treatments included passive loosening exercises of upper and lower limbs, manipulation exercises to reduce spasticity, and exercises to improve trunk control. Neuropsychologists used devices to improve the auditory and visual tracking.
At the first cognitive evaluation, we observed that auditory channel was more responsive than visual channel. During rehabilitative process, multisensory devices were used to stimulate visual and auditory channels such as Neurowave System and, Sniffin Stick test to stimulate olfactory way (Bonanno et al., ) and tactile stimulation to record different tactile responses (hot, cold, rough, smooth, soft). After 2 months of hospitalization, our patient became more responsive by using visual tracking. Our results confirmed that patient had a moderate recovery of consciousness. In fact, clinical scale scores improved especially between the second and third time point (see Table ). Patient was assessed as a minimal responder because he showed self-awareness of the surrounding environment. Although, cognitive and relational profile showed a clinical recovery, motor profile remained unchanged. |
pmc-6520366-1 | The first case is a 40-year-old female from the Duanzhou District, Zhaoqing City, with an onset date of 22 December. The patient was admitted to the hospital on 28 December and is now in critical condition. The second case is a 25-year-old male from Shenzhen City, Guangdong Province, who developed symptoms on 1 January. The patient was admitted to the hospital on 4 January and is now cured and left the hospital. He has a history of visiting a live poultry market. He worked in his father’s restaurant and had no other medical problems in his past history.
The two patient’s conditions deteriorated rapidly, and one-sided pneumonia progressed to two-sided pneumonia. When patient 1 arrived at the designated hospital, her condition was critical. She was given invasive mechanical ventilation and was transferred to the intensive care unit immediately. Other treatments, including gastric protective treatments, methylprednisolone, intravenous immunoglobulin and fluid infusion, were given. The complications and treatments are shown in Table . Despite active treatment, her condition continued to deteriorate. Fifty days after admission, she died from multi-organ failure. The male patient arrived at the designated hospital with 80% oxygen saturation, and then, he received noninvasive ventilator-assisted breathing. He was also given treatments, including gastric protective treatments, methylprednisolone, intravenous immunoglobulin and fluid infusion. His condition improved gradually, and 14 days after the illness onset, he was discharged. |
pmc-6520427-1 | A 51-year-old man was admitted to the Department of Surgery at our hospital for complaints of fever and anorexia. There was no abdominal pain, nausea, or abdominal distension. He had a high fever, anorexia, tachycardia, and tachypnea. He had no comorbidities or surgical history. The white blood cell count, liver enzymes, and C-reactive protein level were elevated. Abdominal ultrasonography showed a hypoechoic lesion with a maximum diameter of 40 mm in the left lobe of the liver (Fig. ). Abdominal contrast-enhanced computed tomography (CT) revealed a lesion with coexisting low- and high-density areas in segment III of the liver. The lesion was adjacent to the stomach antrum and had a maximum diameter of 55 mm, with enhancement at the edge. The lesion contained air and a high-density linear object measuring about 24 mm (Fig. a, b). We suspected a liver abscess secondary to gastric perforation caused by a foreign body. The patient underwent abscess drainage and removal of the foreign body using five-port laparoscopic surgery. Adhesions had formed between the liver and reticulum due to inflammation (Fig. a). We confirmed pus leakage, performed lysis of adhesions, and found a fish bone inside the reticulum (Fig. b–d). The fish bone was removed laparoscopically. We lavaged the abscess cavity with saline. The operation was completed with the insertion of a drain inferior to the left lobe of the liver. There was no bile leakage from the abscess cavity. The total operative time was 62 min, and the total intraoperative blood loss was 20 ml. The pus culture showed the presence of Streptococcus anginosus, which matched the result of the blood culture. We used meropenem until postoperative day 10. The clinical course was uneventful, and the patient was discharged on postoperative day 11. When the patient was discharged, we changed the antibiotic treatment from meropenem to a combination of potassium clavulanate and amoxicillin hydrate according to the indications of the blood culture. On outpatient postoperative follow-up, he had no complaints and laboratory tests were normal. Abdominal ultrasonography indicated that the hypoechoic region was smaller (Fig. ). There has been no recurrence. |
pmc-6520477-1 | Forty-six-year-old male admitted to the Clinic of Tropical and Parasitic Diseases, Poznan, Poland, because of persistent low-grade fever, lymphadenopathy, joint pains, and watery diarrhea lasting for 5 weeks. Prior to the admission, he was hospitalized in the Internal Ward, but no tentative diagnosis was established, except chronic tonsillitis (he was classified among surgery treatment). Previously, he was also suffered from skin Streptococcus pyogenes and Staphylococcus aureus mixed infection. No history of previous travels.
On admission to the Clinic, he was afebrile. Physical examination revealed presence of cervical and axillar lymphadenopathy and increased bowel movements. Blood tests confirmed an internal inflammation (elevated levels of ESR 36 mm/h, CRP 31.8 mg/l, WBC 9.17 G/l). Because of diarrhea, stool examination was performed. Bacteriological culture according to enteropathogenic bacteria (Salmonella spp., Shigella spp., E.coli ETEC, Yersinia spp., Campylobacter spp., Clostridium difficile) was negative. However, the modified Ziehl–Neelsen staining smears revealed presence of huge amount of Cryptosporidium spp. Oocysts, i.e., 20–50 oocysts/10 fields (magnification 1000×), which according to Castro-Hermida et al. () was classified as a massive infection (Fig. ). Moreover, elevated fecal calprotectin concentration was detected. Fecal occult blood test (FOBT) was also positive. Electrophoresis showed increased levels of α2-, β2-, and γ-globulins (15.6/6.9/16.8%) and shortage of albumins (47%).
Because of the massive cryptosporidiosis, the immunodeficiency diagnosis was initiated. Architect CMIA HIV test was negative, HIV-RNA (HIV Cobas TaqScreen MPX version 2.0-Roche) was also negative, normal CD4 count (507/mm3), Treponema RPR test negative, ELISA Toxoplasma gondii (IgM and IgG) negative, ELISA Toxocara IgG negative, HBsAg absent, anti-HCV absent, normal immunoglobulin levels (IgA 1.89 g/l, IgG 11.43 g/l, IgM 0.79 g/l). Colonoscopy (after the patient’s permission) detected numerous small afts and shallow ulcers as well as mucosa scarifications.
The histopathological preparations showed incorrect large intestine mucosa architecture with persistent inflammation within submucosa caused by eosinophils and disseminated in lamina propria neutrophils (cryptitis, Fig. ).
The patient was treated with tilbroquinolonum, trimethoprim/sulphametoxazole (2 × 960 mg), Saccharomyces boulardii, spiramycin (3 × 3.0 mln u.). The patient improved and the parasitic stool test on the last day (after 2 weeks of hospitalization) was negative. Patient was admitted to the Clinic once again after 2 weeks because of relapse of the symptoms (15–20 watery stools, abdominal pain, fever up to 39 °C). The stool examination revealed massive Cryptosporidium spp. infection, i.e., 20–30 oocysts/10 fields (magnification 1000×, Castro-Hermida et al. ). The presence of other parasites was excluded. Combined therapy with Pyrimethamine (1 × 75 mg), Azythromycin (1 × 500 mg), and Nitazoxanide (2 × 500 mg) was initiated. After 10-day treatment, Cryptosporidium eradication was obtained with confirmation after 3 weeks. In control colonoscopy, no signs of inflammatory process were found. |
pmc-6520477-2 | Fifty-six-year-old male hospitalized for 14 days in the Clinic of Tropical and Parasitic Diseases, Poznan, Poland, because of persistent diarrhea, 4–5 watery stools without blood and mucus and fever up to 39 °C. The symptoms appeared 1 year before the admission to the hospital. The patient was treated symptomatically and empirically with amoxicillin, ciprofloxacin, cefuroxime with periodic improvement. He also lost 10 kg of weight. His medical history of chronic diseases and previous travels was unremarkable.
The physical examination performed on admission revealed presence of numerous enlarged, solid cervical lymphatic nodes, tenderness to palpation in the left subcostal region, and intensive bowel movements. Laboratory findings confirmed acute inflammatory response: increased level of CRP (79.3 ng/l, ESR 47 mm/h, leukocytosis 16.73 G/l), monocytosis (8.4%), neutrocytosis (81.3%), and thrombocytosis (672 G/l). Bacteriological stool tests excluded Salmonella, Shigella, Escherichia coli, and Yersinia spp. infection. Repeated parasitological stool examinations (modified Ziehl–Neelsen staining) revealed presence of numerous Cryptosporidium spp. oocysts, i.e., 20–50 oocysts/10 fields (magnification 1000×; Fig. ) and elevated level of WBCs and RBCs. High level of fecal calprotectin (895 μg/l) in the stool sample indicated acute bowel inflammation. FOBT was positive. Possible immunodeficiency was excluded, as in the case no. 1.
Colonoscopy (after the patient’s permission) performed during hospitalization revealed presence intensive inflammatory mucosal lesions, numerous polyps narrowing the bowel bore, presence of stool-purulent content, and different-shape ulcers (cryptitis). CT scans showed the extraperitoneum lymphadenopathy and intestine wall inflammation (mucosa thickening). Histopathologic sections showed incorrect lamina propria architecture with cryptitis. Chronic, severe inflammation was detected in submucosa. The most abundant type of cells was eosinophils. Disseminated neutrophils, mucosal ulcers covered with granular, and fibrinous changes were also observed.
Combined therapy with trimethoprim/sulphametoxazole and rifaximin was initiated together with symptomatic treatment (20% albumins, iron supplementation, anti-diarrheal agents). The improvement of the clinical condition was observed, and the patient was discharged home.
After 2 months, the patient was admitted to the Clinic for follow-up. Several parasitic stool examinations were negative. Colonoscopy showed only presence of pseudopolyp and only slight mucosal changes. The histopathological section without abnormalities. Complete normalization of laboratory parameters (CRP-8.7 ng/l, ESR–7 mm/h, WBC-10.1 G/l, PLT–473 G/l, iron 82 μg/dl, TIBC 396 μg/dl) was observed. Parasitological stool examination revealed Cryptosporidium eradication. |
pmc-6520477-3 | Forty-six-year-old woman admitted to the hospital for the differential diagnosis of chronic diarrhea. From 1 month, she had several watery, sometimes bloody stools with high fever up to 39°. Laboratory results showed high CRP level (37.1 ng/l), increased ESR (33 mm/h), WBC (9.1G/l). USG of the abdomen cavity did not reveal any abnormalities. Bacteriological stool culture detected Campylobacter jejuni. The patient did not consent to the colonoscopy.
Parasitic stool examination showed massive infection with Cryptosporidium spp. oocysts, i.e., 10–40 oocysts/10 fields (magnification 1000×, Castro-Hermida et al. ). High fecal calprotectin level was also detected (> 1000 μg/g). The FOBT was positive. The immunodeficiency was excluded like in the previous cases. The patient was treated with trimethoprim/sulphametoxazole (2 × 960 mg), azythromycin (1 × 500 mg), rifaximin (2 × 400 mg). After 10-day-treatment symptoms diminished, stool examinations were negative, and the patient was discharged home.
During follow-up visit in out-patient department, she reported numerous loose stools. Parasitological examinations of the stool samples discovered Cryptosporidium oocysts. Treatment with nitazoxanide was initiated (3 × 500 mg for 1 week and 2 × 500 mg for 3 days). After the treatment, parasite eradication was obtained. |
pmc-6520636-1 | A 49-year-old male patient arrived at our emergency department with a painful right inguinal mass. The patient has unremarkable past medical and surgical history. He was afebrile, with vital signs within normal limits. The patient presented with a reducible right inguinal mass in the preceding 6 months, which became irreducible 3 days ago, with associated localized pain. The nonreducible right groin mass persisted, and the pain progressed. Manual reduction failed after moderate sedation. Laboratory tests revealed leukocytosis (12,800 white blood cells/μL). Contrast-enhanced CT of the abdomen and pelvis was performed. The images demonstrated that a dilated appendix with appendicolith and perifocal fat stranding in the right inguinal canal (A–C). A preoperative diagnosis of Amyand’s hernia was highly suspected. After obtaining informed consent, diagnostic transabdominal laparoscopy was performed which disclosing an engorged appendix incarcerated in the right inguinal internal ring (A). After laparoscopic lysis of the adhesions, the appendix was reduced. The hernia sac was checked with no residual component (B). Because acute appendicitis seemed to be a straightforward diagnosis grossly without suspicion, laparoscopic appendectomy was performed. Pathological examination confirmed acute appendicitis, with fibrinopurulent exudate and a 0.3 cm fecalith impaction. Concerning about the infectious complications of immediate mesh repair, we decided to perform hernia repair in an elective setting, that is, after resolution of localized infection and inflammation. Postoperative course was smooth, and the patient was discharged 2 days later. Elective laparoscopic total extraperitoneal (TEP) hernioplasty with 10 × 15 cm polypropylene mesh was performed 3 months later. No adverse events occurred postoperatively. |
pmc-6520723-1 | A 62-year-old Japanese man with no past medical history noticed pain and a soft tissue mass on the medial side of the right thigh. Due to gradual worsening of the pain, the patient consulted a local doctor. On physical examination, the patient was not found to have any nerve palsy. On MRI (a–e), a soft tissue mass, arising in the multi-compartment areas of the posterior thigh muscles and intertwining with the sciatic nerve, was revealed, and the patient was referred to the Department of Orthopaedic Surgery, Nagoya City University Hospital. A needle biopsy was performed and diagnosed as undifferentiated pleomorphic sarcoma (UPS) by the Division of Pathology of Nagoya City University Hospital (). The histological grade was 3 according to the Fédération Nationale des Centres de Lutte Contre le Cancer (FNCLCC) grading system. The patient also underwent computed tomography (CT) and thallium scintigraphy (f) for the assessment of the viability of the tumor and detection of metastases; however, there was no evidence of any metastatic lesions. In this study, to assess the accumulation of the tracer, the tumor to background ratio (TBR) is calculated using the formula TBR = (L−B)/B, for accumulation of the tracer at lesion (L) with background (B) []. TBR was calculated by experienced radiologists who were independent of this study.
As a preoperative therapy, RHC was proposed for surgical down-staging. The patient was fully informed of the possible adverse events associated with RHC and agreed with our treatment strategy. Before RHC, the artery reservoir was inserted into the superficial femoral artery, and simultaneously, the inferior gluteal artery, partially feeding the tumor, was embolized using a coil. Intra-artery chemotherapy was simultaneously performed with hyperthermia. Concomitant radiotherapy was administered to the primary site for a total of 40 Gy (2 Gy × 20). Details of the RHC procedures are described by Aiba et al. [], ( and ). Due to grade 2 kidney failure (CTCAE 1.0 criteria), the amount of antitumoral agent was decreased to 80% after three cycles. Furthermore, the white cells count decreased (grade 3) at three cycles, but spontaneously recovered to within the normal range. Anorexia and nausea (grade 2) sometimes occurred during RHC, but no additional fluid therapies or tubal feedings were required.
After five courses of RHC, the uptake of contrast agent on the MRI was attenuated (a–c) and the efficacy of chemotherapy was considered to be a partial response (based on modified RECIST criteria []). Furthermore, the uptake of the tracer was significantly decreased (d). Thus, it was presumed that the micro-invasion of the tumor to the sciatic nerve was attenuated. Also, if the contact between the tumor and the sciatic nerve was not too tight, it would be possible to preserve the sciatic nerve without leaving micro-residue of the tumor using the ISP. For this reason, wide resection using the ISP procedure was proposed for the preservation of the sciatic nerve. |
pmc-6520783-1 | A 72-year-old man underwent left upper division segmentectomy because a ground-glass neoplasm was found in the left upper lobe (A). The lesion was pathologically diagnosed as adenocarcinoma in situ (AIS), TisN0M0, stage 0 (B). AIS is a well-differentiated adenocarcinoma, and the survival rate after resection is reportedly 100% []. Three indels in the noncoding region of the SFTPB gene were detected (). The detection of noncoding indels in a case of such early, well-differentiated lung cancer suggested that noncoding indel mutations occur in the early phase of tumor evolution. |
pmc-6520783-2 | A 79-year-old woman was found to have a massive tumor measuring 90 mm in diameter in the left lower lobe and underwent left lower lobectomy combined with resection of the thoracic wall and diaphragm (C). Histologically, the lesion was diagnosed as squamous cell carcinoma, pT4N0M0, stage IIIB (D). An indel in the noncoding region of the SFTPB gene was detected ().
Thus, noncoding indels were detected in lesions ranging from early-stage lung cancer, as in case I, to massive and poorly differentiated cancer, as in this case. |
pmc-6520783-3 | A 78-year-old woman was found to have a massive tumor measuring 78 mm in diameter in the left upper lobe and underwent left upper lobectomy and chest wall resection. In this case, with pleomorphic carcinoma, histopathologically, the cancer was composed of papillary adenocarcinoma and sarcomatoid cancer, pT3N0M0, stage IIB (A–C).
Whole-exome sequencing analysis of each component was performed, and phylogenetic analysis showed that the separate components shared several common mutations and that the sarcomatous component ramified from the carcinomatous component in the early phase of the evolutionary process and accumulated several mutations that were different from those of the carcinomatous component (D). In terms of the noncoding indel mutations, the same noncoding indel was detected in both the adenocarcinoma and sarcomatoid cancer components (), and phylogenetic analysis revealed that this indel mutation occurred in the common trunk in the early phase of tumor evolution (E). |
pmc-6521294-1 | A 29-week-old pregnant woman, 36 years old, resident of Itaboraí, metropolitan region of RJ, started a febrile illness with vomiting, arthralgia, headache, and epigastria on 03/16/2013. She was admitted to a hospital in RJ on 03/17/2013, presenting a leukocyte count of 13,000/mm³, hematocrit of 33.7%, platelet count of 276,000/mm³, and a positive result for a DENV non-structural protein 1 (NS1) antigen. She was dismissed and requested to return within 48 h for a new evaluation. On 03/22/13, the patient returned to the health unit with pain in the lower limbs, vaginal bleeding, a leukocyte count of 14,000/mm³, a hematocrit of 34.3%, and a platelet count of 112,000 mm³. She was hospitalized in the intensive care unit (ICU). The following day, she had intense bleeding, blood pressure of 13 × 10, vomiting with blood, a leukocyte count of 445,000/mm³, a hematocrit of 26.5%, and a platelet count of 56,000/mm³. The ultrasound revealed a stillborn; vaginal delivery was performed. The fetus was detached from the placenta and an autopsy was performed. Fragments of liver, spleen, brain, lung, and placenta were collected and sent to the Flavivirus Laboratory, FIOCRUZ on 03/27/2013 for case investigation. After vaginal delivery, she remained in the ICU, but died 11 days later, on 04/02/13. No autopsy was performed at that time. The case was classified as Dengue with Complications (DCC), according the criteria established by the Brazilian Ministry of Health in 2000. DCC was established to define severe dengue cases that did not meet the 1997 WHO criteria for dengue hemorrhagic fever (DHF) and dengue shock syndrome (DSS). |
pmc-6521405-1 | A 39-year-old female with history of asthma, obesity, and menorrhagia presented to the hospital with one-week history of exertional shortness of breath. In addition to dyspnea, she reported right lower extremity swelling and pain, which started 4 days prior to this presentation. The patient denied chest pain, palpitations, recent weight loss, known history of personal or family history of blood clots, history of miscarriages, and recent travel. Her only medication was oral contraceptive pill for menorrhagia.
She was hypoxic requiring 2 liters of supplemental oxygen via nasal cannula to maintain oxygen saturation >90%, with respiratory rate 18 breaths per minute. She was mildly tachycardic with a heart rate of 108 bpm and blood pressure was 144/90, and the temperature was 36.6°C. The physical examination was significant for morbid obesity and right lower extremity swelling without sings of phlegmasia cerulea dolens. Troponin I level was normal, BNP was elevated at 704 pg/ml (reference 0-100 pg/ml), 12 lead ECG showed sinus rhythm 95 bpm with S1Q3T3 pattern (): Chest X-ray was unremarkable. Wells' score was 7.5 (3 points for clinical signs and symptoms of deep vein thrombosis, 3 points of PE being the most likely diagnosis, and 1.5 points for heart rate > 100 bpm) rendering high chance of PE; therefore, CT pulmonary angiogram (CTPA) was performed (). It demonstrated saddle type pulmonary embolus with extension into multiple segmental and subsegmental branches in the bilateral upper and bilateral lower lobes along with right ventricular (RV) strain (RV end-diastolic diameter was increased with RV to left ventricular (LV) diameter ratio >1) and mild dilatation of main pulmonary artery (35mm, normal is < 29 mm) [–]. CTPA also showed a linear band of low density extending within the left atrium and into the left ventricle most compatible with thrombus.
The patient was started on unfractionated heparin infusion. Transthoracic echocardiogram (TTE) was performed to further assess the intracardiac clot burden, the RV strain and to identify right-to-left shunt. TTE () showed a large mobile mass straddling the interatrial septum with elements in both atria, extending to and past the mitral valve and prolapsing into the left ventricle during diastole. This was highly suggestive of a communication between the atria consistent with large patent foramen ovale or atrial septal defect. Moderately dilated RV (RV/LV diameter ratio >1) with severely impaired RV systolic function, moderate RA dilation, moderate to severe tricuspid regurgitation, pulmonary artery systolic pressure of 73 mmHg and systolic flattening of the interventricular septum were also noted. All these findings were consistent with RV strain caused by PE. Lower extremity venous doppler ultrasound showed unstable occlusive extensive thrombus within the right femoral vein extending through the right popliteal vein.
Because of large clot burden as well as impending paradoxical embolization, the patient underwent emergent open pulmonary embolectomy, removal of right and left atrial thrombi, and PFO closure (). Intraoperative transesophageal echocardiogram (TEE) confirmed the TTE findings (): because of the presence of an unstable extensive lower extremity venous thrombus an inferior vena cava filter was also placed. The patient tolerated the surgery well and was discharged from the hospital on oral anticoagulation with warfarin. Six month later during her follow-up visit repeat TTE demonstrated improvement in RV dilation, TV regurgitation, and PA pressure (PA systolic pressure was reduced to 26 mmHg). |
pmc-6521420-1 | A 25-year-old male, labourer, inhabitant of Hyderabad, India, presented with history of abnormal behaviour, delusions, hallucinations, and aggression beginning a few hours prior to presentation. The onset was acute and progressive in nature. According to his wife, he suddenly stood up and started picking at his clothes and searching for something in a drawer; at the same time he was not responding to her calls. He was also reported to be sniffing like a dog. When she intervened, he became aggressive and physically assaulted her. He was confused and did not seem to respond to the surroundings. No history of fever, fall, head trauma, or substance abuse was reported. On examination, patient's vitals, and all systems including neurological system were normal. Rapport could not be established as the patient appeared confused and perplexed and was not responding to any questions. He appeared to be agitated and hallucinating as he was talking to self. He was admitted and on further evaluation it was revealed that although his speech was sparse, on many occasions he said God was talking to him and he could also see Him; hence he has the power to do anything. He only intermittently recognized family members. His orientation to time, person, and place was lost on many occasions. However, there were bouts of aggressive behaviour which was nondirected and seemed purposeless. All routine investigations were within normal limits including electrolytes, computed tomography (CT) brain, and electroencephalogram (EEG). The patient was diagnosed as acute transient psychotic disorder according to the tenth revision of the International Classification of Mental and Behavioural Disorders (ICD 10) criteria. He was given intramuscular, haloperidol 10mg IM twice for 2 days. Improvement was noticed in terms of sleep, control of aggression, and orientation. Surprisingly, all the psychotic symptom improved within 2 days. Later he was switched to oral olanzapine 10mg OD due to mild extrapyramidal side effects and was discharged after 3days. He returned after 15 days with complaints of abnormal behaviour. According to his wife he was aggressive, entered a restaurant, and broke the furniture. Patient had no memory of aggressive behaviour. On history taking it was revealed that the present episode lasted only for few minutes. The nature of symptoms as described by patient is feeling of twisting in the abdomen and felt that the bubbles of rain had become very large since it was raining that day. At the same time, he had a thought of infidelity about his wife and did not remember the subsequent episode of aggression which was reported by the wife. On examination the vitals were normal and mental status examination (MSE) seemed to be absolutely normal. Moreover, on further enquiry the wife recollects several such episodes of short-lived abnormal behaviour for the last two years. Video EEG monitoring was recommended in the higher level medical care facility, which he could not do due to financial constraints. He was started on oral carbamazepine 200mg BID on clinical suspicion of CPS, which was later increased gradually to 1000mg per day. Simultaneously olanzapine was tapered off. The patient has been maintained on 1000mg oral carbamazepine per day for the last 2 years with no relapse in the symptoms. |
pmc-6521464-1 | A 45-year-old man presented with stage II buccal SCC involving the labial commissure (Fig. ). Under general anesthesia, the A-EF was drawn along the nasolabial fold, creating a triangular total thickness flap; the flap was used to reconstruct the defect in the commissure following tumor resection and radical neck dissection (Fig. ). The inner and outer dimensions of the A-EF skin paddle were 1.8 × 3.0 and 1.8 × 2.5 cm, respectively. At the 3-month follow-up, the esthetic result for the cheek and lip was excellent, the orbicularis oris function was rated 3 (can suction water with a straw), and the speech function was rated 3 (normal speech) (Fig. ). At 23 months, the patient had local recurrence and underwent salvage surgery. |
pmc-6521464-2 | A 43-year-old man presented with stage III buccal SCC involving the labial commissure. Under general anesthesia, he was placed on his side at an angle of approximately 45° with the head and neck extended moderately. Tumor resection and neck dissection were performed in this position. The folded extended SFIF based on the transverse cervical vessels and incisions for the tumor resection are shown in Fig. . A foldable flap with a skin paddle including inner (7 × 5 cm) and outer (7 × 8 cm) linings for reconstructing the full cheek defect and labial commissure were created by dissecting the skin in the flap bilaterally (Fig. ). The flap was pulled through a tunnel to reach distant cheek defects; the medial portion of the flap was used for the buccal mucosa and the distal portion was used for the skin of the cheek following tumor resection and neck dissection (Fig. ). The donor site was closed directly. At the 6-month follow-up, the esthetic result for the cheek and lip was satisfactory; the orbicularis oris function was rated 2 (can suction some water with a straw), and the speech function was rated 2 (intelligible speech) (Fig. ). The patient was alive with no evidence of disease at 26 months. |
pmc-6521464-3 | A 63-year-old man presented with stage IV buccal SCC involving the labial commissure (Fig. ). Under general anesthesia, a folded PMMF was used to reconstruct the defect in the commissure (Fig. ). The flap was based on the thoracoacromial vessels and pulled through a tunnel to reach the distant cheek defect following tumor resection, with a partial maxillotomy plus marginal mandibulotomy and radical neck dissection. At the 4-month follow-up, the esthetic result for the cheek and lip was satisfactory; the orbicularis oris function was rated 2 (can suction some water with a straw) and the speech function was rated 2 (intelligible speech) (Fig. ). The patient was alive with no evidence of disease at 22 months. |
pmc-6521464-4 | An 81-year-old woman presented with stage IV recurrent buccal SCC involving the labial commissure (Fig. ). Under general anesthesia, a folded extended vertical lower TIMF based on the transverse cervical vessels was raised with the patient in the lateral prone position. The flap was designed to follow the course of the transverse cervical vessels, ensuring that the center of the long axis of the flap was between the vertebral column and the medial border of the scapula (Fig. ). The flap was raised from its midpoint and proceeded in a medial to lateral direction, preserving the upper part of the trapezius muscle; a tunnel was made in the upper part of the trapezius muscle. A foldable flap with a skin paddle including inner (7 × 5 cm) and outer (7 × 10 cm) linings for reconstructing the full cheek defect and labial commissure was created by dissecting the skin in the flap bilaterally (Fig. ). The donor area was closed primarily. After tumor resection, a partial maxillotomy plus marginal mandibulotomy and radical neck dissection were performed (Fig. ). The distal portion of the flap was turned to serve as the inner lining or oral mucosa, and the medial portion for the outer lining or skin (Fig. ). At the 3-month follow-up, the esthetic result for the cheek and lip was satisfactory; the orbicularis oris function was rated 2 (can suction some water with a straw), and the speech function was rated 2 (intelligible speech) (Fig. ). The patient was alive with no evidence of disease at 27 months. |
pmc-6521468-1 | Local Institutional Review Board approval was obtained, and the patient’s guardian gave their written, informed consent to publish this case.
A 49-year-old Japanese woman with a history of depression was found in an unconscious state by her husband after ingesting an unknown amount of boric acid, mirtazapine (1950 mg), and sennosides (780 mg) and transported to a local medical facility. She had unstable circulation so she was transported to our Department of Acute Critical Care Medicine. On arrival, her vital signs were as follows: Glasgow Coma Scale, E1V1M1; blood pressure, 45/13 mmHg; heart rate, 190 beats per minute; and body temperature, 37.0 °C. She had systemic diffuse erythema. There were no other physical findings. Electrocardiography (ECG) showed atrial fibrillation tachycardia. She received tracheal intubation with mechanical ventilation. A cardiac echocardiogram depicted diffuse hypokinesis of wall motion with an ejection fraction of 20%. Chest roentgenography revealed no findings. Whole-body computed tomography to evaluate her brain condition, residual drugs in her stomach, and accompanying lesions indicated bilateral dorsal lung consolidation, suggesting aspiration []. The results of an arterial blood gas analysis, cell blood count, and biochemical study are shown in Table . Soon after, she exhibited pulseless electrical activity; spontaneous circulation was obtained by advanced life support. However, she experienced repeated episodes of pulseless electrical activity, and percutaneous cardiopulmonary bypass was required due to unstable circulation. An emergency coronary angiogram was negative. She was admitted to an intensive care unit and underwent additional continuous hemodialysis filtration due to acute kidney injury with anuria. She remained in a deep coma state without sedation. On the second hospital day, cardiac motion ceased on the echocardiogram, but her atrial fibrillation rhythm continued. As her husband strongly wished to continue these treatments, we selected to continue them until cardiac standstill. However, cardiac motion was obtained again on the fourth hospital day and stable spontaneous circulation with sinus rhythm was obtained on the sixth hospital day; thus, percutaneous cardiopulmonary bypass was withdrawn. She exhibited hair loss. She remained in a persistent unconscious state; thus, tracheostomy was performed on the seventh hospital day. Sufficient urinary flow was obtained, and her potassium level remained within the normal limits; thus, continuous hemodialysis filtration was withdrawn on the tenth hospital day. Head magnetic resonance imaging showed signal change in the bilateral white matter and caudate nuclei, suggesting hypoperfusional cerebral ischemia. As she developed right leg dry necrosis, which was induced by cannulation for percutaneous cardiopulmonary bypass, leg amputation was performed on the 22nd hospital day. She also developed bilateral corneal ulcers, which was possibly due to boric acid poisoning.
Stable spontaneous respiration was finally obtained on the 30th hospital day; thus, mechanical ventilation was withdrawn. Systemic erythema gradually subsided with desquamation. She displayed spontaneous eye opening and spontaneous movement of her extremities; however, she could not respond to any commands. She was transferred to a local medical facility in a persistent unconscious state on the 39th hospital day. A subsequent examination revealed that her serum concentrations of boric acid and mirtazapine on arrival were 560.49 mg/L and 1270 ng/mL, respectively (Fig. ). She could speak simple words for commands and swallow food but was unable to walk; thus, at 6 months after collapse, she was totally dependent in her activities of daily living. |
pmc-6521477-1 | The patient is a 50-year-old female who had a 4-year history of left lumbar radiculopathy which was precipitated from a lifting injury that did not resolve following L4-5 microdiscectomy. She had previously been unsuccessfully managed with epidural steroid injections and multimodal analgesics. A nerve conduction study was unremarkable for any pathology. She was diagnosed with failed back surgery syndrome and counseled regarding her treatment options, including spinal cord stimulation. She elected to pursue spinal cord stimulation with the Nevro Senza HF10 system. As part of her evaluation for SCS placement, the patient was seen at the behavioral medicine clinic and underwent presurgical psychological testing and evaluation by a board-certified psychologist. Her psychological assessment profile suggested a high likelihood of a good postoperative outcome. She had a history of depression and anxiety, both of which were stable and under medical management. The patient underwent an uneventful SCS trial with near 100% relief of her back and leg pain and wished to proceed with permanent implant. Her permanent placement procedure was uneventful with leads placed at the same levels as her trial leads (.) with the tip of the right lead at the top of the T8 vertebral body and left lead tip at the mid T9 vertebral body. The device was activated in the post-anesthesia care unit with bipole setting on electrodes 10 and 11.
The first day following her procedure, the patient noted a buzzing or a chirping sound predominately in her left ear as well as vertigo, intermittent involuntary left facial twitches, and perioral numbness, all of which were new to her. She also reported nausea, vomiting, and diarrhea, which she has attributed to anxiety in the past. She was subsequently evaluated by ENT around 4 weeks postoperatively and underwent an audiological evaluation with no abnormal findings. There was concern that the tinnitus could be related to her stimulator. She also later complained of implanted pulse generator (IPG) site pain and stinging sensations. Attempts at reprograming her SCS system with a bipole settings on leads 4 and 5 failed to resolve her ongoing neurologic symptoms. The patient was subsequently advised to deactivate her system to see if her symptoms would improve. Two days after deactivation of her system, the patient reported complete resolution of her symptoms. She was again evaluated and spine radiographs were performed which demonstrated unchanged position of her SCS leads. She was not interested in reactivating her system to assess for return of symptoms. The SCS system was subsequently explanted. At the time of explant, there were no visible defects in the SCS system. |
pmc-6521491-1 | An 8-year-old boy was admitted to the Emergency Room of Santa Maria della Misericordia hospital, Perugia, Italy, on February 10, 2018 because he had suffered from severe diarrhoea (> 6 stools per day), vomiting (3 episodes per day) and high fever (> 39.0 °C) over the previous two days. He did not have unusual travel, dietary, or animal contact and did not receive any doses of the RV vaccine. In the Emergency Room, the child was drowsy, body temperature was 38.7 °C, heart rate 170 beats/min, respiratory rate 50 breaths/min, and blood pressure 85/45 mmHg. Severe dehydration was evident. The weight was 2.5 kg lower than that evaluated several days before disease onset, revealing a 10% loss. The oral mucosa was very dry, eyes sunken and skin liftable in persistent folds. Moreover, no urine was found in the bladder after catheterization. Laboratory tests revealed low serum concentrations of both potassium (2.7 mEq/L) and sodium (128 mEq/L), confirming hypo electrolytic gastroenteritis. Glycaemia was lower than the normal (47 mg/dL). Kidney function tests showed a relevant pre-renal failure, with creatinine at 2.48 mg/dL and urea at 133 mg/dL. Severe acute gastroenteritis was diagnosed according to the Ruuska and Vesikari criteria for the definition of severity of RV diarrhoeal episodes [], and the child was hospitalized.
After hospitalization, faecal samples for bacteria and virus identification were collected. Cultures for all the bacterial pathogens usually associated with acute severe gastroenteritis were negative, while an enzyme immunoassay for the detection of RVA (Ridascreen® Rotavirus, R-Biopharm AG, Germany) was positive. A sample of faeces was used for reverse transcription-polymerase chain reaction and nucleotide sequencing of the infecting RV. Moreover, tests were performed to evaluate host immune system function, including total immunoglobulin concentration, neutrophil function and lymphocyte immunophenotyping; however, no abnormalities were revealed.
Intravenous fluids were immediately administered according to the World Health Organization recommendation [], and the response was rapid. Diarrhoea progressively reduced, vomiting disappeared, and oral feeding was resumed the day after admission. Blood pressure, heart rate, and respiratory rate returned to normal values within 24 h as it was for diuresis. Pre-renal failure disappeared after 48 h. Weight returned to initial values within one week.
Management of the case was approved by the Ethics Committee of Umbria Region, Perugia, Italy (2018-PED-03), and parents provided informed written consent. The patient’s parents also provided informed consent for the publication of this case report.
Total RNA was extracted from 140 μL of 10% faecal suspensions in distilled water using the Viral RNeasy Mini Kit (Qiagen/Westburg, Segrate, Italy), according to the manufacturer’s instructions. RNA was eluted in 60 μL of RNase-free water and stored at − 80 °C until use. After an initial step of denaturation, the viral RNA was subjected to retro-transcription (RT) using the Invitrogen Superscript III reverse transcriptase kit (Life Technologies, Monza, Italy) with a single cycle at 37 °C for 60 min and 95 °C for 5 min. The obtained DNA was then used as a template for PCR amplification of VP7 (primers Beg9-End9) and VP4 (primers Con3-Con2) segments [, ]. The reactions were performed with the Invitrogen Platinum Taq kit (Life Technologies, Monza, Italy), following the manufacturer’s instructions. RVA genotyping was carried out by a multiple semi-nested PCR using a mixture of primers specific for G- and P-types, as previously described for genotype assignment []. Retro-transcription and all PCR reactions were performed following slightly modified EuroRota-Net protocols [].
Since the low amount of DNA obtained after the first round PCR, for nucleotide sequence analysis, a second nested PCR was carried out using VP7-F, VP7-R, VP4-F, and VP4-R primers, resulting in a 881 bp product for VP7 and a 663 bp product for VP4 [, ]. Nucleotide sequencing of amplified genes was performed at Eurofins Genomics (Ebersberg, Germany) using primers used for the second PCR. In addition, the amplification and nucleotide sequencing of all the RVA genomic segments was performed. Briefly, the RT-PCR was conducted by using specific primers for each gene segment, and nucleotide sequencing was performed at Eurofins Genomics using the same primers included in the RT-PCR reactions. The nucleotide sequences obtained were analysed and corrected with ChromasPro2.23 software (Technelysium, Queensland, Australia). Nucleotide and amino acid sequence similarity searches were performed using the BLAST (Basic Local Alignment Search Tool) server on the GenBank database of the NCBI (National Center for Biotechnology Information, National Institute of Health, Bethesda, MD).
The assignment of genotypes for all the 11 gene segments was performed with the “RotaC v2.0 - classification tool for rotaviruses group A”. Multiple sequence alignments and phylogenetic tree construction were performed with MEGA6 software [], applying the maximum-likelihood method and using the Tamura3 + G substitution model. The sequences obtained in this study are available in GenBank () under the accession numbers listed in Table .
The phylogenetic trees revealed relatedness between the VP7 and VP4 genes of the PG2 G3P[8] RVA Italian strain and those belonging to recent G3P[8] RVAs detected worldwide. The G3 VP7 belonged to the equine-like G3 lineage and shared the highest nucleotide sequence identity (99.8%) with the previously identified equine-like G3 (Fig. ). Moreover, it was significantly different from the G3 strains detected previously in Italy and included in the phylogenetic tree within the G3-3c and 3d lineages. The P[8] VP4 revealed a similar clustering pattern as that observed for the VP7. Strain PG2 was closely related to the same strains highlighted by the VP7 analysis, with the P[8] Italian strains previously detected in the same sampling area (in combination with G1 and G4 VP7) grouped separately, despite sharing the same lineage 3 in this case (Fig. ).
Finally, the PG2 RVA strain clustered in a branch different from that of the VP7 gene of Rotateq® (18.5% nucleotide difference and 7.2% amino acid difference) and in a lineage different from those of the VP4 specificities included in the Rotateq® and Rotarix® vaccines (6.3 and 9.6% nucleotide difference and 5.8 and 10.3% amino acid difference, respectively). The comparison of the deduced amino acid sequences revealed only 4 substitutions (T87A, N213 T, K238D, D242A) across the VP7 epitopes (Fig. ).
Strain PG2 showed the G3-P[8]-I2-R2-C2-M2-A2-N2-T2-E2-H2 genotype, revealing a complete genotype 2 (DS-1 like) genomic backbone []. All the gene segments investigated revealed the highest nucleotide sequence identities (98.5 to 99.8%) with equine-like G3P[8] RVA strains detected in Brazil in 2015 (Table ). |
pmc-6521521-1 | We report a 44-year-old previously healthy woman a month ago, who experienced a transient numbness of her right sided face and arm. She was referred to our unit with a possible diagnosis of MS or of a systemic autoimmune disease due to small brain lesions in MRI. For years, she had been complaining of intermittent weakness of her left arm, but it had been attributed to psychosomatic problems.
Upon presentation, the neurological examination revealed indifferent plantar reflexes and jerky deep tendon reflexes. Her brain MRI revealed various lesions of hyperintensity in T2 and Fluid Attenuated Inversion Recovery (FLAIR) sequence, in the subcortical white matter of the hemispheres and also periventricular, with mild linear cortical, enhancement of the left parietal lobe. Cerebrospinal fluid analysis and laboratory tests for inflammatory-autoimmune diseases were normal.
However, a more thorough analysis of brain MRI revealed that lesions were mainly in the border zone of anterior cerebral artery (ACA) and middle cerebral artery (MCA) with no evidence of restricted diffusion (). Given the watershed distribution of brain lesions, further evaluation with magnetic resonance angiography (MRA) of the brain was recommended to exclude possible cerebrovascular disease. Brain MRA showed a significant stenosis in the proximal segment of MCA and ACA bilaterally and collateralization mainly around M1 and A1 segments, findings typically seen in MMD (). Digital subtraction angiography (DSA) of neck and head confirmed diagnosis (Figures and ). Antiplatelet therapy started, but two days later, she suddenly developed global aphasia and right hemiparesis (National Institutes of Health Stroke Scale/ NIHSS 6) and brain MRI revealed acute infarct in the distribution of the left MCA (Figures and ). Thrombolysis was not performed due to increased risk of hemorrhage in MMD and the gradual improvement of the patient. At discharge, she improved with a residual aphasia and right facial paresis (NIHSS 3). |
pmc-6521524-1 | A 62-year-old Japanese male with a horseshoe kidney was referred to our hospital for further examination of left hydronephrosis. The patient was obese with a body mass index of 32.0 kg/m2 (height 170 cm, body weight 92 kg) and had undergone medical treatment for hypertension. Laboratory examination results showed mild renal dysfunction with a serum creatinine level of 1.21 mg/dL and estimate glomerular filtration rate of 48.2 mL/minute. Computed tomography (CT) revealed a left lower ureteral mass near the ureteral orifice, resulting in renal hydronephrosis (Figures and ). Furthermore, three-dimensional (3-D) CT imaging showed a normal renal vascular system, while an aberrant renal artery located just below the root of the inferior mesenteric artery (IMA) was found to supply the bilateral sides of the lower part of the horseshoe kidney (). In addition, urine cytology findings were positive for malignancy. Based on these findings, we diagnosed a left lower ureteral tumor associated with a horseshoe kidney.
In June 2017, laparoscopic left nephroureterectomy and bladder resection procedures were performed with 5 laparoscopic ports, with the port schema shown in . Using an intraperitoneal approach, we cut the peritoneum and dislocated the intestine to approach the renal helium. After visualizing the left renal vascular system, the main renal artery supplying the left side of the upper part of the horseshoe kidney and left branch of the aberrant renal artery passing under the IMA were ligated by use of a Hem-o-lok® polymer clip and divided, after which the left renal vein was ligated and divided in the same manner (). Next, we were able to observe the renal isthmus using an NIRF system (da Vinci Xi FireFlyTM®) at 1 minute after intravenous administration of 1 mL of ICG (Diagnogreen 0.25%; Daiichi Pharmaceutical, Tokyo, Japan). Strong ICG fluorescence was observed in the dominant region of the right part of the kidney, while no fluorescence was noted in the left kidney region (). These findings indicated that blood supply to the left side of the kidney was completely blocked, which was more apparent as compared to white light images (). Subsequently, the heminephrectomy was started under the assistance of NIRF imaging using cold scissors. During excision, visualization with white light and the NIRF was switched at the discretion of the operator in order to confirm the plane of excision between the right and left parts of the kidney (Figures and ). Adequate hemostasis from the resected area could be obtained by electrocautery bipolar coagulation and use of a TacoSil® tissue sealing sheet, with no suturing required (Figures and ). Following completion of the laparoscopic heminephrectomy, the patient was placed in a supine position and partial resection of the bladder was performed. The total operation time was 5 hours 21 minutes and blood loss was 410 mL (obtained in urine from the bladder). A histopathological examination revealed an invasive urothelial carcinoma (grade 2>1, INFβ, pT2a, ly0, v0, u-lt0, RM0). There were no perioperative complications and the patient was discharged 9 days after surgery. |
pmc-6521531-1 | A 38-year-old male patient, who was suffering from psoriasis vulgaris for 2 years, paid a visit to our centre. Preliminary examination of the patient showed that numerous erythematous plaques with numerous silvery scales present all over the scalp including the area behind the ears. The severity of the disease was assessed to be 28 on Psoriasis Scalp Severity Index (PSSI), calculated by the standard method which combines the severity (erythema, induration, and desquamation) and percentage of affected area.
Adipose tissue was collected from a healthy volunteer by lipoaspiration by a plastic surgeon under the aseptic conditions in the O.T. About 100 ml of fat was aspirated out from the waist area and collected in a sterile container. The fat tissue contacting stem cells was processed in a biosafety laminar airflow chamber. MSCs were isolated from adipose tissue by standard enzymatic digestion method with 0.1% collagenase type I. Following the centrifugation, the resulting pellet was cultured in DMEM medium (Invitrogen, Paisley UK) supplemented with 10% foetal bovine serum (FBS) and 1% penicillin/streptomycin, at 37°C in humidified atmosphere containing 5% CO2. The media were changed after every 3 days. About 5×106 MSCs of passage 2 were seeded in each T175 culture flask (n=10) containing 30 ml of DMEM medium supplemented with 10% FBS. MSCs were confirmed with spindle shaped morphology and free from any contamination () using a phase-contrast microscope. When cells attained 90% confluence at passage 2, the culture media were replaced with serum-free DMEM. After 72 h of incubation, resulting MSC-CM was collected, centrifuged at 2000 rpm for 5 min to remove the cell debris, filtered through 0.22-μm filter, and then concentrated (10 times) by ultrafiltration using centrifugal filtering units with a cut-off value of 3 kDa (Amicon Ultra-15; Millipore, MA), according to the manufacturer's instructions. The concentrated MSC-CM was aliquoted and stored at -20°C until use. MSC-CM was topically applied on the afflicted areas once a day over a period of one month. Clinical parameters like severity, changes, and clearance of psoriatic plaques were monitored at regular intervals.
Numerous psoriatic erythematous plaques with adhering silvery scales over the different regions of the scalp were observed before the treatment regimen started. In general, the number of the scales declined significantly within 2 weeks of topical application of MSC-CM. Interestingly, clearance of silvery scales and severity of psoriatic plaques were completely abolished within one month of the treatment (). The PSSI score reduced to 0 from 28 and regression of the disease continued for 6 months of follow-up. The patient did not take any other medication during the follow-up period of six months and led an improved quality of life without any adverse side effects. |
pmc-6521550-1 | 39-year-old male with no significant past medical history presented to the urgent care for some discomfort in chest. He was referred to emergency department for suspected pulmonary embolism workup. Computed tomography (CT) scan () of the chest with contrast revealed no pulmonary embolism. However, the striking abnormality on the CT was bilateral upper lobe mass with bilateral nodules in the perilymphatic distribution with multiple enlarged mediastinal and hilar lymphadenopathy. He was referred to a pulmonologist for outpatient workup and eventually the patient underwent a positron emission tomography (PET) scan. The PET scan showed numerous FDG avid lymph nodes in mediastinum along with bilateral lung nodules. Also present were lesions in the right scapular () and the left sacral bone ().
The patient then underwent mediastinoscopy for biopsy of the lymph nodes. Review of the final pathology of the original biopsy specimen at our institution demonstrated noncaseating granulomas suggestive of sarcoidosis. However due to uncertainty of the bone lesions he was sent for biopsy of bone lesions which were reported positive for sarcoid like pattern. The patient received oral prednisone 40 mg/day therapy and subsequently had complete recovery of his symptoms. On a 3-month follow-up, repeated CT scan showed resolving mass and lymph node size. |
pmc-6521550-2 | 38-year-old male presented to emergency department for right abdominal pain. CT abdomen showed right renal stone along with left lung lower lobe opacity. He was referred to outpatient pulmonary clinic where he was sent for CT scan chest () which again showed the left lower lobe opacity with mediastinal lymphadenopathy. A PET scan was done revealing multiple sites of pathologic uptake suspicious for biologic tumor activity in left lower lobe node, mediastinal lymph node, and lesion in left iliac bone (). He was sent to a cardiothoracic surgeon wherein the patient underwent mediastinoscopic guided biopsy of the lymph nodes along with biopsy of the iliac bone lesion. Both biopsy results were positive for sarcoid like pattern. Subsequently, he also underwent left lower lobe lesion biopsy which also appeared positive for sarcoid. |
Subsets and Splits
Exclude ER emergencies
Retrieves 100 descriptions that do not contain the terms 'ER' or 'emergency', providing a basic filter of the dataset.